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Clinical and epidemiological comparison of patients affected by palmoplantar plaque psoriasis and palmoplantar pustulosis: A case series study
Abstract
Summary Background In 2007 the International Psoriasis Council proposed that palmoplantar pustulosis (PPP) should be considered a separate condition from psoriasis, despite the presence of certain phenotypes common in both diseases.
Objectives To describe and compare demographic and clinical characteristics among patients with PPP and palmoplantar plaque psoriasis.
Methods This was a retrospective case series study from 2005 to 2010. The following data were obtained: age, sex, family history, smoking habits, nail involvement, joint involvement, disease duration, lesion morphology (plaque or pustular), histological diagnosis, comorbidities, and Physician’s Global Assessment (PGA) score for extrapalmoplantar lesions. The sample size calculation indicated that 80 patients, 40 patients for each group (palmoplantar plaque psoriasis and PPP) were needed to see clinically relevant differences between groups.
Results Ninety patients were selected, 51 with palmoplantar plaque psoriasis and 39 with PPP. No statistically significant differences were registered between patients affected by PPP and palmoplantar plaque psoriasis as regards age at onset of the disease (48 vs. 44 years; P = 0·4), disease duration (6 vs. 10 years; P = 0·1), family history of psoriasis (28% vs. 33%; P = 0·7), concomitant arthritis (26% vs. 25%; P = 1·0), or smoking habits (54% vs. 41%; P = 0·2). We observed a female predominance (P = 0·01) and a lesser frequency of nail involvement (P = 0·03) in patients affected by PPP.
Conclusions Our data suggest a close relationship between PPP and psoriasis. The existing data concerning epidemiology, clinical presentation, genetics, histopathology and pathogenesis do not permit a clear distinction between these two entities, which seem to coincide in many aspects. PPP appears to have a marked predilection among female smokers.
... [1,2] NPPP and PPP are distinct from psoriasis vulgaris, although all conditions share similar phenotypic expressions. [1][2][3][4][5][6][7][8]17] Unlike psoriasis vulgaris, palmoplantar psoriasis is a rare condition. [17,18] It is estimated that the prevalence of PPP is 0.005 to 0.12% [19,20], whereas the prevalence of NPPP is 0.12% to 0.36% [17]. ...
... [1][2][3][4][5][6][7][8]17] Unlike psoriasis vulgaris, palmoplantar psoriasis is a rare condition. [17,18] It is estimated that the prevalence of PPP is 0.005 to 0.12% [19,20], whereas the prevalence of NPPP is 0.12% to 0.36% [17]. ...
... [1][2][3][4][5][6][7][8]17] Unlike psoriasis vulgaris, palmoplantar psoriasis is a rare condition. [17,18] It is estimated that the prevalence of PPP is 0.005 to 0.12% [19,20], whereas the prevalence of NPPP is 0.12% to 0.36% [17]. ...
Introduction:
Palmoplantar psoriasis is classified as nonpustular palmoplantar psoriasis (NPPP) or palmoplantar pustulosis (PPP).
Objective:
We sought to shed light on the phenotypic diversity of nail psoriasis and conducted a thorough study of the dermoscopic features of nail lesions from patients with NPPP and PPP.
Methods:
A prospective study included 35 patients with NPPP and 20 patients with PPP who had findings of nail psoriasis in at least three of their nails. Demographic and clinical data, such as general characteristics, history, a thorough dermatological examination, and musculoskeletal assessment, were recorded. Each patient had their nails evaluated with videodermoscopy. The statistical analysis was carried out using SPSS software, including descriptive and analytical statistics.
Results:
Dermoscopic patterns of nail lesions in both groups differed from those seen in psoriasis vulgaris. Trachyonychia was the most common dermoscopic pattern among NPPP patients. Nail bed pustules were found in 75% of the PPP patients, and they were the most common dermoscopic finding identifying PPP nail lesions. In comparison to the NPPP group, patients with PPP had a higher frequency of psoriatic arthritis (PsA) and higher palmoplantar psoriasis and nail psoriasis severity scores. In the PPP group, all patients with PsA had pustules.
Conclusions:
Nail psoriasis has a heterogeneous presentation and various clinical manifestations in NPPP, PPP, and psoriasis vulgaris. The differences in dermoscopic patterns of nail lesions among NPPP, PPP, and psoriasis vulgaris reflect the diseases' distinct characteristics. A thorough dermoscopic examination of the nails may reveal information about both the underlying pathophysiological pathways and the overall course of psoriasis.
... Palmoplantar pustulosis (PPP) is a chronic refractory inflammatory skin disease characterized by repeated eruptions of sterile pustules on the palms and/or soles [1]. It presents as chronic or recurrent erythematous hyperkeratotic plaques, with involvement of the palms (5-15%), soles (17-18%), or both (67-77%) [2,3]. National prevalence of PPP in Japan between April 2010 and March 2011 was 0.12% [4], which is substantially higher than the 0.01-0.05% ...
... Epidemiological studies indicate a clear preponderance of women: 62-83% of PPP patients are women; moreover, a majority (54-95%) are current or former smokers [2,3,6,[12][13][14]. The average age of disease onset is > 40 years [2,3,[12][13][14]. ...
... Epidemiological studies indicate a clear preponderance of women: 62-83% of PPP patients are women; moreover, a majority (54-95%) are current or former smokers [2,3,6,[12][13][14]. The average age of disease onset is > 40 years [2,3,[12][13][14]. It is widely known that focal infections and smoking are involved in the development and exacerbation of PPP, and their management is important to treat PPP [15]. ...
Palmoplantar pustulosis (PPP), a refractory skin disease characterized by repeated eruptions of sterile pustules and vesicles on palms and/or soles, involves interleukin-17 pathway activation. Brodalumab, a fully human anti-interleukin-17 receptor A monoclonal antibody, is being investigated for use in PPP treatment.
The aim was to assess the efficacy and safety of brodalumab in Japanese PPP patients with moderate or severe pustules/vesicles.
A phase 3, randomized, double-blind, placebo-controlled trial was conducted between July 2019 and August 2022, at 41 centers in Japan. Patients aged 18–70 years with a diagnosis of PPP for ≥ 24 weeks, a PPP Area Severity Index (PPPASI) score of ≥ 12, a PPPASI subscore of pustules/vesicles of ≥ 2, and inadequate response to therapy were included. Participants were randomized 1:1 to receive brodalumab 210 mg or placebo, subcutaneously (SC) at baseline, weeks 1 and 2, and every 2 weeks (Q2W) thereafter until week 16. Changes from baseline to week 16 in the PPPASI total score (primary endpoint) and other secondary skin-related endpoints and safety endpoints were assessed.
Of the 126 randomized patients, 50 of 63 in the brodalumab group and 62 of 63 in the placebo group completed the 16-week period. Reasons for discontinuation were adverse event (n = 6), withdrawal by patient/parent/guardian (n = 3), progressive disease (n = 3), and lost to follow-up (n = 1) in the brodalumab group and Good Clinical Practice deviation (n = 1) in the placebo group. Change from baseline in the PPPASI total score at week 16 was significantly higher (p = 0.0049) with brodalumab (least-squares mean [95% confidence interval {CI}] 13.73 [10.91–16.56]) versus placebo (8.45 [5.76–11.13]; difference [95% CI] 5.29 [1.64–8.94]). At week 16, brodalumab showed a trend of rapid improvement versus placebo for PPPASI-50/75/90 response (≥ 50%/75%/90% improvement from baseline) and Physician’s Global Assessment 0/1 score: 54% versus 24.2%, 36.0% versus 8.1%, 16.0% versus 0.0%, and 32.0% versus 9.7%, respectively. Infection was the dominant treatment-emergent adverse event (TEAE); the commonly reported TEAEs were otitis externa (25.4%/1.6%), folliculitis (15.9%/3.2%), nasopharyngitis (14.3%/4.8%), and eczema (14.3%/12.9%) in the brodalumab/placebo groups, respectively. The severity of most TEAEs reported was Grade 1 or 2 and less frequently Grade ≥ 3.
Brodalumab SC 210 mg Q2W demonstrated efficacy in Japanese PPP patients. The most common TEAEs were mild infectious events.
NCT04061252 (Date of Trial Registration: August 19, 2019)
... Palmoplantar pustulosis (PPP) is a chronic inflammatory and recurrent skin condition characterized by pustules on the palms and soles [1]. In Western countries, the prevalence of PPP is estimated to be between 0.01 and 0.05% [1][2][3]. ...
... Palmoplantar pustulosis (PPP) is a chronic inflammatory and recurrent skin condition characterized by pustules on the palms and soles [1]. In Western countries, the prevalence of PPP is estimated to be between 0.01 and 0.05% [1][2][3]. Although the International Psoriasis Council proposed that PPP should be considered a discrete skin disorder from psoriasis (PsO) [4], there has been continued debate on whether PPP is a variant of PsO, as [ 10% of patients affected by psoriasis present with PPP as well. ...
... Although the International Psoriasis Council proposed that PPP should be considered a discrete skin disorder from psoriasis (PsO) [4], there has been continued debate on whether PPP is a variant of PsO, as [ 10% of patients affected by psoriasis present with PPP as well. [1,[4][5][6][7][8] Currently, no cure or approved standard of care exists for PPP, and defining the optimal course of treatment for patients with PPP has been challenging [9,10]. Available treatment options focus mainly on symptom management and primarily include topical and systemic therapies, phototherapy, and targeted biologics; however, evidence on the risks and benefits of these treatments in this population is limited [11]. ...
Limited information exists on the epidemiology, treatment, and burden of palmoplantar pustulosis (PPP) and defining the optimal course of treatment remains challenging without approved targeted treatments in most countries. Here, we describe the clinical and demographic characteristics, treatments received, and negative health outcomes experienced among patients with PPP in the United States (US) and Germany.
Retrospective cohort study between 2016 and 2021 using data from the US Merative™ MarketScan® Research Database and IQVIA™ German Disease Analyzer. Adult patients with PPP (ICD-10-CM L40.3) were followed from the date of their first qualifying PPP diagnosis and continued until the earlier of disenrollment or end date of database, during which treatment patterns and incidence rates of negative health outcomes were assessed. Treatment patterns included adherence, non-persistence, discontinuation, re-initiation, switching, and combination therapy.
The prevalence of PPP was 0.005% and 0.065% in the MarketScan database and German Disease Analyzer, respectively, with 1629 and 3866 patients meeting the inclusion criteria. Most patients were female (71.3%, 67.8%), with mean (SD) age of 54.1 (11.7) and 56.9 (14.3) years, respectively. One year post index, most patients received topical treatment (77.4%, 65.3%), but non-persistence and discontinuation were high. Oral and biologic treatments displayed higher levels of adherence, particularly apremilast and tofacitinib among oral treatments and TNF inhibitors and IL-23 inhibitors among biologics. Rates of negative health outcomes were higher among patients not receiving treatment post-index compared with those receiving treatment post-index across both databases, regardless of prior treatment history.
Establishing treatment guidelines remains an unmet need for patients with PPP and could improve quality of life by reducing the occurrence of negative health outcomes. The findings from this study may provide insight into the effectiveness of current treatment options for patients with PPP and can be leveraged to support the development of treatment guidelines.
... PP is characterized by scaly erythematous plaques, with or without pustules, and is a chronic, inflammatory, and debilitating cutaneous condition. It can be further classified into subtypes based on its various morphologic features [2]. ...
... Occasionally, extrapalmoplantar lesions may also be observed in PPP. PP can coexist with psoriasis involving other body areas, and PPP is also associated with plaque psoriasis in approximately 20% of cases [2,4]. Despite their localized presentation, both PP and PPP can cause significant physical and functional impairment, greatly impacting patients' quality of life [2,5]. ...
... PP can coexist with psoriasis involving other body areas, and PPP is also associated with plaque psoriasis in approximately 20% of cases [2,4]. Despite their localized presentation, both PP and PPP can cause significant physical and functional impairment, greatly impacting patients' quality of life [2,5]. Additionally, both conditions can be recalcitrant to conventional treatments, posing therapeutic challenges for dermatologists [6]. ...
The comparative efficacy of biologics and small-molecule inhibitors in treating palmoplantar psoriasis (PP) and palmoplantar pustulosis (PPP) remains uncertain.
The aim was to perform a systematic review and network meta-analysis (NMA) to compare the efficacy of biologics and small-molecule inhibitors for the treatment of PP and PPP.
MEDLINE, Embase, and Cochrane Central Register of Controlled Trials were searched for eligible studies from inception to May 13, 2023. This NMA was conducted and reported following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses Extension Statement for Network Meta-Analyses guidelines. Frequentist random-effects models NMA was performed with the surface under the cumulative ranking curve calculated for ranking. Our primary outcome was the proportion of patients achieving a clear/minimal Palmoplantar Psoriasis/Pustulosis Physician Global Assessment score (PPPGA 0/1 or PPPPGA 0/1) response at 12–16 weeks. Secondary outcomes consisted of the percentage of overall improvement in palmoplantar score and of improvement ≥ 75%, at 12–16 weeks.
The study comprised a total of 29 randomized controlled trials (RCTs), involving 4798 psoriasis patients with palmoplantar diseases. For PP, 16 RCTs with nine different treatments, including adalimumab, apremilast, bimekizumab, etanercept, guselkumab, infliximab, ixekizumab, secukinumab, and ustekinumab were included for the analysis. In the NMA of PP, secukinumab 300 mg ranked highest (odds ratio [OR] 33.50, 95% confidence interval [CI] 4.37–256.86) in achieving PPPGA 0/1, followed by guselkumab 100 mg (OR 18.68, 95% CI 10.07–34.65). In the case of PPP, seven RCTs with six treatments, including apremilast, etanercept, guselkumab, imsidolimab, spesolimab, and ustekinumab, were included for the analysis. In the NMA of PPP, although no treatment demonstrated a significant difference compared to placebo in achieving PPPPGA 0/1, guselkumab 100 mg showed the greatest statistically significant improvement in the palmoplantar score (weighted mean difference 31.73, 95% CI 19.89–43.57) as a secondary outcome.
Among all available biologics and small-molecule inhibitors, secukinumab 300 mg and guselkumab 100 mg had the most favorable efficacy in treating PP and PPP, respectively.
... Although PPP is often described as a variant of palmoplantar pustular psoriasis, epidemiological and biological studies have suggested that the 2 diseases have different etiologies (Asumalahti et al, 2003). PPP has a prevalence of 0.12% in Japan (Kubota et al, 2015), higher than that in Western countries (0.01e0.05%) (Brunasso et al, 2013;de Waal and van de Kerkhof, 2011). Patients with PPP have an extremely high smoking rate (69e90% in males and 44e60% in females) (Iwaki et al, 2022;Kondo et al, 2021). ...
... Patients with PPP have an extremely high smoking rate (69e90% in males and 44e60% in females) (Iwaki et al, 2022;Kondo et al, 2021). Previous case-control studies with a small sample size suggested that smoking is associated with an increased PPP risk (Brunasso et al, 2013;Miot et al, 2009). However, no large-scale epidemiological studies have investigated the risk factors for PPP. ...
... Few case-control studies have investigated the relationship between smoking and PPP risk (Brunasso et al, 2013;Miot et al, 2009). This study has validated the association between smoking and PPP risk. ...
... Our study revealed a female predominance for PPP and a mean age of onset of 42.38 years, similar to previous reports. 2,3,[5][6][7][8][9][10][11][12][13] We found that PPP onset is most common in palmar areas (41.1%); however, both palmar and plantar involvement was the most common presentation (60.1%) at the time of recruitment. Two previous reports 14,15 described a similar pattern, indicating that PPP typically starts on either palmar or plantar areas (64% and 72%, respectively) before involving both. ...
... In our study, 42% of patients reported nail involvement, which is within the range of 10%-76% reported in previous studies. 3,[5][6][7][9][10][11][12]18,19 The prevalence of PsA or arthralgia also varies in previous literature, ranging from 8% to 48%. 3,[5][6][7][9][10][11][12]19 We found that 11.2% of patients with PPP had concomitant PsA, as diagnosed by rheumatologists, which is a low proportion relative to the previous studies mentioned above. ...
... 3,[5][6][7][9][10][11][12]18,19 The prevalence of PsA or arthralgia also varies in previous literature, ranging from 8% to 48%. 3,[5][6][7][9][10][11][12]19 We found that 11.2% of patients with PPP had concomitant PsA, as diagnosed by rheumatologists, which is a low proportion relative to the previous studies mentioned above. Discrepancies in the prevalence of nail involvement and PsA can be attributable to several factors, including differences in study designs (retrospective vs. crosssectional), differences in the definition of PsA or arthralgia, overdiagnosis or underdiagnosis of nail findings and joint symptoms, and genetic or racial/ethnic differences. ...
Background
Palmoplantar pustulosis (PPP) is a rare, chronic, inflammatory skin disease characterised by sterile pustules on palmar or plantar areas, and data on PPP are scarce.
Objectives
To investigate the PPP clinical characteristics and risk factors for disease severity amongst a large cohort of Turkish PPP patients.
Methods
We conducted a cross-sectional, multicentre study of PPP patients recruited from 21 tertiary centres across Turkey.
Results
A total of 263 patients (165 women, 98 men) were evaluated. Most patients (75.6%) were former or current smokers. The mean Palmoplantar Pustulosis Area and Severity Index (PPPASI) score was 8.70 ± 8.06, and the mean Dermatology Life Quality Index (DLQI) score was 6.87 ± 6.08, and these scores were significantly correlated (r = 0.524, p < 0.001). Regression analysis showed that current smoking was significantly associated with increased PPPASI scores (p = 0.028). Co-existing psoriasis vulgaris (PSV) was reported by 70 (26.6%) patients. The prevalence of male sex, palmar PPP onset incidence, disease duration, DLQI score, nail involvement, and psoriatic arthritis prevalence were significantly increased among PPP patients with PSV. Of the patients, 18 (6.8%) had biologic therapy-induced paradoxical PPP, and these patients had significantly increased mean DLQI scores and PSA prevalence (r = 0.026, p = 0.001).
Conclusions
Our data suggested that smoking is a risk factor for both PPP development and disease severity, PPP patients with PSV present distinct clinical features, and patients with biologic therapy-induced paradoxical PPP have reduced quality of life and are more likely to have PSA.
... Traditionally PPP has been classified as a subgroup of psoriasis, as it is often seen in combination with psoriasis vulgaris. 1,2 Smoking, infection and certain drugs trigger or exacerbate the disease. 3 Many people who have PPP experience reduced quality of life, and one study reported that PPP is associated with greater impairment of quality of life than moderate-to-severe psoriasis. ...
... 1,6 PPP is more frequent in women than in men, with reported male-to-female ratios of 0Á21-0Á52, 5 and the median age at onset has been found to be between 45 and 65 years. 2,6 Prevalence estimates of PPP using populationbased administrative databases have recently been determined in Denmark and Japan. 1,7 In the current study we used longitudinal healthcare register data covering the total Swedish population (~9Á9 million in 2015). ...
... We found that PPP was more common in women than in men (male-to-female ratio of 0Á26), which is in line with estimates from other studies (range 0Á21-0Á53). 2,7,14,20 Psoriasis vulgaris occurred in 17% (n = 2500) of the PPP population. Most patients (63%) were diagnosed with only code L40Á0, followed by only L40Á9 (27%). ...
Background
Generalised pustular psoriasis (GPP) is a severe form of pustular psoriasis with generalised eruption of sterile pustules, often along with systemic symptoms. There is a scarcity of population-based estimates of GPP prevalence and incidence.
Objectives
To estimate i) the prevalence and incidence of GPP in the Swedish general population and ii) the prevalence of psoriasis vulgaris within the GPP population.
Methods
We identified cases (2004-2015) with one ICD-10 diagnostic code (base case) for GPP within the Swedish National Patient Register, which covers inpatient and outpatient secondary care. Cases were linked to the Swedish Total Population Register and the point prevalence was estimated as on 31 December 2015. In two alternative analyses we changed case definitions to: 1) requiring two visits (strict case 1), 2) requiring two visits of which one within dermatology/internal medicine (strict case 2).
Results
The base case point prevalence of GPP was estimated to 9.1/100 000 (women=11.2, men=7.0) and the annual prevalence in 2015 was estimated to 1.53/100 000. Among the GPP population, 43% also had a psoriasis vulgaris code. The incidence of GPP in 2015 was estimated to 0.82/100 000 (women=0.93, men=0.74). The criteria used had an impact on prevalence and incidence estimates; prevalence strict case 1) 3.8/100 000 and incidence strict case 1) 0.42/100 000.
Conclusions
Results indicate that the estimated GPP population in Sweden is within the range of previous published estimates. However, the estimates were sensitive to employed GPP case criteria. The findings enhance demands for studies using validated diagnostic algorithms.
... PPP involves the palms and/or soles exclusively and presents with sterile pustules, background erythema, hyperkeratosis and scaling [5,49]. Cutaneous involvement favors the thenar, hypothenar and central areas of the palms, and the soles at the corresponding levels [8,50]. Coalescence of pustules and hyperpigmentation after resolution are common features. ...
... PPP is regarded as a common dermatosis in Japan where its classical presentation is consistent with Andrews' type A-PPP [51]. PPP typically affects middle aged women, especially smokers [8,13,50]. Possible comorbidities are arthro-osteitis, metabolic syndrome and thyroid disease [4,10,50]. The Japanese literature reports that around 30% of PPP patients develop musculoskeletal manifestations [9]. ...
... PPP typically affects middle aged women, especially smokers [8,13,50]. Possible comorbidities are arthro-osteitis, metabolic syndrome and thyroid disease [4,10,50]. The Japanese literature reports that around 30% of PPP patients develop musculoskeletal manifestations [9]. ...
Pustular psoriasis (PP) is a clinicopathological entity encompassing different variants, i.e., acute generalized PP (GPP), PP of pregnancy (impetigo herpetiformis), annular (and circinate) PP, infantile/juvenile PP, palmoplantar PP/palmoplantar pustulosis, and acrodermatitis continua of Hallopeau (ACH), which have in common an eruption of superficial sterile pustules on an erythematous base. Unlike psoriasis vulgaris, in which a key role is played by the adaptive immune system and interleukin (IL)-17/IL-23 axis, PP seems to be characterized by an intense inflammatory response resulting from innate immunity hyperactivation, with prominent involvement of the IL-36 axis. Some nosological aspects of PP are still controversial and debated. Moreover, owing to the rarity and heterogeneity of PP forms, data on prognosis and therapeutic management are limited. Recent progresses in the identification of genetic mutations and immunological mechanisms have promoted a better understanding of PP pathogenesis and might have important consequences on diagnostic refinement and treatment. In this narrative review, current findings in the pathogenesis, classification, clinical features, and therapeutic management of PP are briefly discussed.
... Traditionally PPP has been classified as a subgroup of psoriasis, as it is often seen in combination with psoriasis vulgaris. 1,2 Smoking, infection and certain drugs trigger or exacerbate the disease. 3 Many people who have PPP experience reduced quality of life, and one study reported that PPP is associated with greater impairment of quality of life than moderate-to-severe psoriasis. ...
... 1,6 PPP is more frequent in women than in men, with reported male-to-female ratios of 0Á21-0Á52, 5 and the median age at onset has been found to be between 45 and 65 years. 2,6 Prevalence estimates of PPP using populationbased administrative databases have recently been determined in Denmark and Japan. 1,7 In the current study we used longitudinal healthcare register data covering the total Swedish population (~9Á9 million in 2015). ...
... We found that PPP was more common in women than in men (male-to-female ratio of 0Á26), which is in line with estimates from other studies (range 0Á21-0Á53). 2,7,14,20 Psoriasis vulgaris occurred in 17% (n = 2500) of the PPP population. Most patients (63%) were diagnosed with only code L40Á0, followed by only L40Á9 (27%). ...
Background
Palmoplantaris pustulosis (PPP) is a chronic relapsing skin condition characterized by sterile pustules on the palm and soles. Population‐based estimates of PPP incidence and prevalence are limited.
Objectives
To estimate the prevalence and incidence of PPP in the Swedish general population and to estimate the prevalence of psoriasis vulgaris among the PPP population.
Methods
The Swedish National Patient Register, covering all inpatient and outpatient non‐primary care for the Swedish population, was used. We identified cases (2004‐2015) with one ICD‐10 diagnostic code (base case) for PPP. The point prevalence estimates at the end of this period (31 December 2015) were obtained by linkage to the Swedish Total Population Register. In sensitivity analyses, we used alternative case defintions:1) requiring two visits, 2) requiring two visits of which one within dermatology/internal medicine.
Results
The base case prevalence of PPP was estimated to be 147/100 000 (women=227, men=68) and the annual prevalence was estimated to 26/100 000 in 2015. Among the PPP population, 17% were registered with a diagnostic code for psoriasis vulgaris. The incidence of PPP in 2015 was estimated to be 12.7/100 000 (women=18.7, men=6.6). The criteria used had an impact on prevalence and incidence estimates; strict case 1) 72/100 000 and 2) 5.4/100 000.
Conclusions
Results indicate that the population‐based prevalence of PPP may be larger than previous estimated. However, the estimates were sensitive to employed PPP case criteria. The findings enhance demands for studies using validated diagnostic algorithms potentially also including data from the primary care setting.
... the 1-year prevalence of PPP in the Usa, europe and Japan has been reported as 0.005-0.12% [4,5], peaking between 44 and 65 years of age [3,4,6]. PPP can co-occur with plaque psoriasis; an estimated 14.2-PPP is a chronic disease [7]. the clinical course of PPP is heterogeneous; lesions can either be persistent or follow a relapsing pattern [7]. in addition, psychiatric, cardiovascular, metabolic, articular and autoimmune comorbidities have been reported with PPP, although the relationship between PPP and systemic diseases is not yet sufficiently studied [5,7,8]. ...
... Furthermore, more than half of patients with PPP reported problems within the DlQi question about treatment (how much of a problem has the treatment for your skin been, for example by making your home messy, or by taking up time?). the distribution of sex was considerably different between patients with PPP and plaque psoriasis, where the proportion of females was 79% vs. 37%, respectively. this was expected as PPP is more common in females than males, whereas severe plaque psoriasis is more common among males [6,20]. Furthermore, as some patients (n = 68) has both PPP and plaque psoriasis, it may be difficult to disentangle the impact of the different psoriasis types on hRQol. ...
Background
Real-world data on health-related quality of life (HRQoL) in palmoplantar pustulosis (PPP) are scarce and few studies have analysed the generic HRQoL.
Objectives
To assess HRQoL using the generic EQ-5D instrument and the Dermatology Life Quality Index (DLQI) instrument in PPP compared to plaque psoriasis.
Methods
Cross-sectional data from PsoReg, the Swedish National Registry for Systemic Treatment of Psoriasis (2006–2021), were examined. The study included 306 patients with PPP, out of which 22% had concomitant plaque psoriasis (n = 68), and 7041 patients with plaque psoriasis only. EQ-5D and DLQI were compared between patients with PPP and patients with plaque psoriasis, overall and stratified by sex. A subgroup analysis compared outcomes for patients with PPP vs. patients with severe plaque psoriasis (Psoriasis Area and Severity Index ≥10). Multiple regression analyses were performed to control for potential confounders (age, sex, comorbidities, lifestyle factors).
Results
Patients with PPP were to a larger extent female (79% vs. 37%, p < .01) and older (mean [SD] age 59.9 [11.9] vs. 50.7 [16.0] years, p < .01) than patients with plaque psoriasis. EQ-5D values were significantly lower (worse) in patients with PPP (mean [SD] 0.622 [0.309]) compared to patients with plaque psoriasis (mean [SD] 0.715 [0.274]). No significant difference was observed compared to patients with severe plaque psoriasis (p = .237). DLQI was comparable in PPP and plaque psoriasis patients (p = .117). In the regression analyses, PPP only and PPP with plaque psoriasis were associated with lower EQ-5D values of 0.065 (p < .01) and 0.061 points (p < .10) compared to plaque psoriasis patients.
Conclusions
PPP had a substantial negative impact on patients’ generic and dermatology-specific HRQoL. Patients with PPP were worse off in terms of generic HRQoL compared with patients with plaque psoriasis when controlling for the impact of potential confounders.
... [4] Palmoplantar psoriasis accounts for 10-12% of all psoriasis cases and is characterized by well-defined erythematous, scaly plaques over palms and soles, and painful fissures over thick plaques. [5] The involvement of palms and soles results in difficulties in performing daily activities and impacts the quality of life disproportionate to body surface area involvement. [6] It is difficult to treat conditions where topical therapy often fails and systemic therapy is required for management. ...
... Palmoplantar psoriasis is a regional variant of psoriasis and is responsible for almost 10-12% of cases of psoriasis. [5] Despite limited body surface area involvement, it causes a severe impact on quality of life due to the involvement of palms and soles, thereby hampering occupational as well as leisure-time activities. Involvement of palms also results in social embarrassment as handshake and other social gestures make patients increasingly conscious of their illness. ...
Background:
Palmoplantar plaque psoriasis is a regional variant of psoriasis, characterized by erythematous, indurated plaques with fissuring over palms and soles. Chronic plaque psoriasis is associated with various comorbidities such as obesity, diabetes mellitus, hypertension, dyslipidemia, metabolic syndrome, and cardiovascular disease. Body surface area involvement is an indicator of psoriasis severity, and most comorbidities are more strongly correlated with severe disease.
Objectives:
To estimate the prevalence of metabolic comorbidities in palmoplantar plaque psoriasis.
Methods:
It is a case-control study involving treatment naïve palmoplantar plaque psoriasis patients and age- and gender-matched healthy controls.
Results:
The study included 100 cases and 100 controls. The mean age among cases and controls was 45.4 ± 11.1 and 43.9 ± 10.3 years, respectively (P: 0.31). The gender ratio among cases and controls was 1.56 (61M: 39F) and 1.94 (66M: 34F), respectively. Comorbidities including metabolic syndrome (P: 0.001), obesity (P: 0.001), diabetes mellitus (P: 0.001), and hypertension (P: 0.001) were more common among cases as compared to controls. The odds of diabetes, metabolic syndrome, hypertension, and obesity in patients with palmoplantar psoriasis were 4.8 (95% CI 2.5-9.3), 3.7 (95% CI 2-6.9), 3.1 (95% CI 1.6-6), and 3.5 (95% CI 1.9-6.4), respectively.
Conclusion:
In this study, we found that palmoplantar plaque psoriasis is associated with metabolic comorbidities. Primary care physicians should screen patients with palmoplantar psoriasis for these comorbidities.
... In contrast to the high effectiveness of new biological drugs in the treatment of psoriasis Vulgaris of the trunk and extremities, in both palmoplantar phenotypes and in the lesions located predominantly on the scalp, these drugs usually have a slow and limited or null effect [3,4]. The marked clinical differences and therapeutic response to the same treatments between these subgroups suggest that psoriasis should be considered as a spectrum of diseases rather than as a single entity [5]. Thus, it is important to unveil potential genetic factors that determine the location and phenotype of psoriasis lesions. ...
... The number of patients in our cohort is limited, but it must be taken into account that we included infrequent subtypes of psoriasis (i.e., palmoplantar pustulosis (PPP) has a prevalence of 0.01-0.05% [44] and hyperkeratotic palmoplantar psoriasis affects approximately 12% of all patients with psoriasis [5]). For this kind of study, power analysis is not straightforward, and the models used for sample size determination are not easily applicable to CNV analysis [45]. ...
Background:
Psoriasis can present different phenotypes and could affect diverse body areas. In contrast to the high effectiveness of biological drugs in the treatment of trunk and extremities plaque psoriasis, in palmoplantar phenotypes and in plaque scalp psoriasis, these same drugs usually have reduced efficacy. Anti-TNF drugs could induce the appearance of palmoplantar pustulosis (PPP) in patients with other inflammatory diseases. The objective of this study is to identify if there are DNA Copy Number Variations (CNVs) associated with these different clinical phenotypes, which could justify the differences found in clinical practice. Moreover, we intend to elucidate if anti-TNF-induced PPP has a similar genetic background to idiopathic PPP.
Methods:
Skin samples were collected from 39 patients with different patterns of psoriasis and six patients with anti-TNF-induced PPP. The CNVs were obtained from methylation array data (Illumina Infinium Human Methylation) using the conumee R package.
Results:
No significant CNVs were found between the different phenotypes and the locations of psoriasis compared. Nevertheless, we found two significant bins harboring five different genes associated with anti-TNF-induced PPP in patients with a different background other than psoriasis.
Conclusions:
Our results may help to predict which patients could develop anti-TNF-induced PPP.
... Если поражение ограничивается областью ладоней и подошв, принято употреблять термин «ладонно-подошвенный пустулез»; если высыпания имеются на других участках тела -«ладонно-подошвенный пустулезный псориаз». Однако, учитывая наличие генетических различий между вульгарным псориазом и ладонно-подошвенным пустулезом, многие авторы поддерживают концепцию, согласно которой ладонно-подошвенный пустулез признается отдельным заболеванием [7,8]. ...
... После 20 сеансов фототерапии клиническая динамика была выраженной (рис. 4Б) -ответ PPPASI 75% (пациентка выписана с индексом PPPASI 3,8). ...
Background: Palmar-plantar psoriasis is characterized by a torpid course and resistance to conventional systemic treatments. Phototherapy is usually considered as an adjuvant treatment of a patient with psoriasis. The potential use of phototherapy as a basic treatment strategy in limited psoriasis, including its plantar-palmar localization, could be of interest.
Aim: To study the efficacy, safety and tolerability of the narrowband phototherapy (UVB 311 nm) in the treatment of different forms of psoriasis with predominant palmar-plantar involvement.
Materials and methods: We retrospectively analyzed the results of treatment of 77 in-patients admitted to the Department of Dermatology for treatment of various types of psoriasis with prevailing palmar and plantar lesions. The main group consisted of 42 patients who were administered combination therapy including topical corticosteroids, hepatic protectors, antihistaminic agents and, in addition, the narrowband phototherapy with a phototherapy device Dermalight 500-1 (Dr. Hnle Medizintechnik GmbH, Germany). The initial radiation doses were set without the determination of the minimal erythema dose, depending on the patient's skin type, in accordance with the guidelines from the manufacturer. At each consecutive session, the dose was increased by 0.060.3 J/cm. The sessions were conducted 5 times a week with a total of 1421 sessions. The mean cumulative dose was 22.8 J/cm. The control group included 35 age-, gender- and psoriasis severity-matched patients who received the same treatments, except the narrowband phototherapy. The treatment efficacy was assessed by changes in the Palmoplantar Pustulosis Area and Severity Index (PPPASI). Clinical results of treatment were evaluated at day 10 after the treatment course had been completed.
Results: No serious adverse events were registered during the treatment. In the patients with psoriasis vulgaris and predominant palmoplantar lesions, receiving the narrowband phototherapy, the PPPASI reduction was higher than in the patients who received only conventional treatment (U-test, p = 0.015). A PPPASI decrease of 50% was observed in 83.3% (25/30) and 60% (15/25) of the patients, respectively. Clinical efficacy criteria were achieved in 66.6% (8/12) of the patients with palmoplantar pustular psoriasis receiving the combination treatment with phototherapy and in 40% (4/10) of the conventionally treated patients in the control group; however, the difference in the distribution of remission achievement was non-significant (U-test, p = 0.123).
Conclusion: The study has demonstrated the efficacy of UVB 311 nm narrowband phototherapy in the treatment of patients with psoriasis with predominant palmoplantar lesions. The results obtained make it possible to recommend the inclusion of the narrowband phototherapy UVB 311 nm at mean cumulative dose of 22.8 J/cm into the standardized set of treatments of patients with psoriasis vulgaris with predominant palmoplantar lesions, not only as an adjuvant technique, but also as the main therapeutic strategy. The role of the narrowband phototherapy UVB 311 nm in the treatment of palmoplantar pustular psoriasis, as well as the dosing regimens of the radiation and determination of the necessary follow-up duration should be the subject of further studies.
... It is the most common reason for visiting the dermatologist and has few effectual treatments (1,2). Although itch sensation can arise from any area of the skin, itchy palms and soles (hairless glabrous skin) are considered the most debilitating and are associated with many dermatological and systemic conditions (3)(4)(5). A few examples include palmoplantar pustulosis (or palmar and plantar psoriasis), a chronic skin disease characterized by inflamed scaly skin and intense itch on the palms and soles that is reported to affect 0.01 to 0.05% of the US population (4); dyshidrosis, a skin condition causing itchy blisters to develop only on the palm and soles that results in an estimated 200,000 US cases per year (5); and cholestatic itch, an intense itching sensation felt in the limbs, and particularly the palms and soles of feet, frequently reported with hepatobiliary disorders (3). In recent years, considerable progress has been made in our understanding of itch with the identification of key molecules and neuronal populations mediating itch in both the peripheral and central nervous system (2,6). ...
... The mechanisms of hairy skin itch have been extensively studied. However, there are numerous dermatological conditions such as contact dermatitis, plantar and palmar psoriasis, tinea capitis, dyshidrosis, and cholestasis associated with glabrous skin itch (3)(4)(5)39). Here, we present evidence showing that different mechanisms exist for hairy and glabrous skin itch. ...
Significance
Chronic itch, associated with dermatologic or systemic diseases, is challenging to treat and significantly affects quality of life. In the past several decades, significant progress has been made to help us understand the mechanisms of itch. Due to the ease of application and analysis, all animal itch behavioral assays utilize pruritogens administered to the hairy skin. However, itch occurs to both hairy and hairless glabrous skin. Many medical conditions such as plantar and palmar psoriasis, dyshidrosis, and cholestasis mainly evoke itch in glabrous skin. We here present evidence demonstrating that distinct neuronal populations are responsible for mediating hairy and glabrous skin itch. This study advanced our understanding of itch and will have significant impact on the clinical treatment of itch.
... Psoriasis is a chronic inflammatory autoimmune disease characterized by an excessive aberrant proliferation of keratinocytes. 1 Palmoplantar psoriasis is a clinical variant of psoriasis affecting palms and soles. 2 The skin lesion of psoriasis is characterized by well circumscribed erythematous, dry scaly plaques of varying sizes covered by silvery white scales having predilection for scalp, extensor surfaces of limbs, umbilical region, palms, soles and nails. 3 There are various clinical variants of psoriasis like chronic plaque psoriasis, inverse psoriasis, generalized pustular psoriasis, erythro-dermic psoriasis and palmoplantar psoriasis. ...
Background
Palmoplantar psoriasis is a clinical variant of psoriasis characterized by well‐defined erythematous desquamating plaques on palms and soles, which may or may not include pustules. Hyperkeratotic lesions of palm and sole commonly include Psoriasis, Eczema and Tinea. These conditions often present with overlapping clinical and histopathological features requiring clinicohistopathological correlation for a conclusive diagnosis. The presence of munro's microabscess or spongiform pustule of kogoj differentiates psoriasis of palm and sole from other hyperkeratotic lesions of palm and sole. The objective of this study was to study the clinical and histopathological profile of palmoplantar psoriasis and correlate clinical diagnosis with histopathological diagnosis.
Method
A hospital‐based, descriptive study was conducted from January 1, 2020, to December 31, 2020. Fifty‐two patients were clinically diagnosed as palmoplantar psoriasis with or without involving other parts of body and routine histopathological evaluation was carried out as per standard protocols.
Result
Clinically diagnosed 52 cases of palmoplantar psoriasis showed varied histopathology with hyperkeratosis (100%), parakeratosis (100%), regular acanthosis (75%), Supra‐papillary thinning (44.2%), spongiosis (65.4%), tortuous vessels in the papillary dermis (78.8%) and mixed inflammatory infiltrates (predominantly lymphocytic‐100%), which were observed to be prominent findings in skin biopsies of our patients. Clinicopathological correlation was achieved in 88.5% of cases.
Conclusion
This study shows clinically diagnosed palmoplantar psoriasis with histopathological features consistent with palmoplantar psoriasis in 88.5% cases. Thus, clinically inconclusive hyperkeratotic lesions with palmoplantar psoriasis can be diagnosed with histopathological correlation improving the therapeutic intervention.
... Associated disorders include pustulotic arthroosteitis (PAO) and Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis syndrome (SAPHO). 5 The presentation of PPP may mimic numerous other conditions such as dyshidrotic eczema, contact dermatitis, pityriasis rubra pilaris, and tinea pedis and manuum. As a result, a thorough history and physical examination are warranted, though additional workup is also doi:10.36849/JDD.7612R1 ...
Palmoplantar pustulosis is a variant of psoriasis and a chronic skin disorder in which pruritic pustular eruptions appear on the palms and soles. It is thought to arise from a variety of genetic and environmental factors, is limited in prevalence, and has proven quite difficult to treat. The symptoms it inflicts on those affected are quite debilitating and the treatment landscape is constantly evolving, thus emphasizing the need for updates of the literature as time passes. Current treatments include topical agents, oral therapies, and phototherapy, amongst other treatments. In this systemic review, we explore newer literature from 2015 to 2022 on various treatment regimens for palmoplantar pustulosis. J Drugs Dermatol. 2024;23(8):626-631. doi:10.36849/JDD.doi:10.36849/7612R1.
... Clinicians have difficulty in accurately diagnosing PP and HE due to the similar histopathologic features of these conditions 6 and because of their similar clinical responses to the systemic treatment of psoriasis with agents such as methotrexate, oral retinoids, and cyclosporine. 7 Because many comorbid diseases including cardiovascular disease, metabolic syndrome, nonalchoholic fatty liver disease, and cancer are more prevalent in psoriasis patients than in the general population, 8 better distinguishment of PP from HE is needed. The aim of our study was to distinguish PP, HE, and HHD histologically. ...
A novel category of spindle cell tumors characterized by Neurotrophic Tyrosine Receptor Kinase (NTRK) rearrangements with a dual immunoreactivity for S-100 and CD34 has emerged in the last years as a distinct entity among soft tissue neoplasms. These genetic alterations lead to the continuous activation of NTRK genes, driving tumorigenesis and offering a unique prospect for targeted therapy. We herein present a rare case of NTRK3-rearranged spindle cell tumor with a hitherto unreported gene fusion involving the exon 14 of NTRK3 with the exon 2 of EML4, arising in the head & neck region. Tumor occurred in a 45-year-old patient who presented with a painful nodule in the oral mucosa. Due to the possibility of personalizing the treatment strategy for such tumors, pathologists should be aware of this emerging group of spindle cell tumors to promptly recognize them even when they occur in uncommon locations, including the oral cavity.
... 14 The thenar or hypothenar eminences and the central palm are common sites of involvement in PPP. 15 Eriksson et al noted 69% of PPP had involvement of both palm and sole unlike our study which had either palm or sole involvement in all cases of PPP. 16 Nail involvement was noticed in 14% to 33% in various studies. ...
Background: Palmo-plantar pustulosis (PPP) and palmoplantar psoriasis with pustules (PPso)/Palmoplantar pustular psoriasis are entities that present with chronic cyclical eruptions of vesicles and pustules of palms and soles, which were initially considered to be similar but was defined as separate entities by the international psoriasis council in 2007. Individuals with PPP are at increased risk of psoriasis vulgaris, psoriatic arthritis, autoimmune thyroid disease and 90% of pustulosis patients can have psoriasis-like rash at some stage of the disease. Aim was to assess the clinical, histopathological and dermoscopic features of PPP and palmoplantar psoriasis with pustules. Methods: A cross sectional analysis of all patients who presented to us over 1 year with vesicles and/or pustules of hands and/or feet and were diagnosed with PPP and palmoplantar psoriasis with pustules/palmoplantar pustular psoriasis, both clinically and histologically, were done. The severity was analysed using palmoplantar pustulosis area severity index (PPPASI) and the clinical, histological and dermoscopic features were noted. Results: Twenty-four patients were categorized into 2 groups of 12 each, with a male predominance in both the groups. Majority (66.6%) of PPP belonged to the age group of 20-40 years whereas 83% of PPso belonged to >40 years of age. Dermoscopic findings noted in both groups were translucent yellow areas, yellow/red-brown globules, dotted regularly arranged vessels, yellow crust and globules following dermatoglyphics. Conclusions: The study, though with limited sample size, highlights few clinical, histopathological and dermoscopic features which helps in differentiating the two groups.
... Subtypes of the disease include plaque, guttate, pustular, erythrodermic, palmoplantar, nail, and inverse psoriasis. Plaque psoriasis is the most common subtype, affecting approximately 80% of patients with psoriasis (Brunasso et al, 2013). Guttate psoriasis is characterized by an acute onset of multiple, small red papules and plaques ranging from 0.5 to 1.5 cm commonly associated with a preceding viral upper respiratory illness. ...
We have previously identified that a structural membrane protein Caveolin-1 (Cav1) is involved in the regulation of aberrant keratinocyte proliferation and differentiation. The aim of this study was to elucidate the role of Cav1, Caveolin-2 (Cav2), and Cavin-1 in the pathogenesis of psoriasis vulgaris and between psoriasis subtypes. We utilized human biopsies from validated cases of psoriasis vulgaris (n = 21) at the University of Miami Hospital and compared the expression of Cav1, Cav2, and Cavin-1 by immunohistochemistry staining with that in normal healthy age-/sex-/location-matched skin (n = 15) and chronic spongiotic dermatitis skin samples (as control inflammatory skin condition) and quantified using QuPath. Distinct subtypes of psoriasis included guttate, inverse, nail, plaque, palmoplantar, and pustular. All biopsy samples exhibited a trend toward downregulation of Cav1, with nail, plaque, and palmoplantar psoriasis exhibiting the most pronounced effects. Only nail and pustular psoriasis samples exhibited significant downregulation of Cav2 and Cavin-1, suggesting Cav1 to be the main caveolar contributor to the pathogenesis of psoriasis. Together, these data support caveolae as pathophysiological targets in nail and pustular psoriasis, whereas Cav1 seems to be a general biomarker of multiple subtypes of psoriasis.
... [8][9][10][11] Contrarily, palmoplantar pustulosis shows a clear female predominance. 9,10,12 Small studies show that guttate, pustular, and localized plaquetype lesions might be more common in women than in men. 13 Genital involvement is reported more frequently in men than in women, especially in anogenital locations (59% in men vs 39% in women), 14 and less intense a difference in the intertriginous region (19% in men vs 17% in women), practically equal. ...
Background
The prevalence of psoriasis is similar between men and women; however, evidence exists of sex- and gender-related differences in disease expression, impact, coping, and needs of patients with psoriasis. These differences are essential and should be considered in clinical practice and research.
Objective
To compile available evidence on sex- and gender-related differences in psoriasis, identify the most critical gaps in clinical practice and research, and use it to propose strategies for improved clinical practice.
Methods
Six European dermatologists selected the topics to consider according to their relevance in the dermatology setting with the support of methodologists. Evidence on sex- and gender-related differences was obtained by a scoping review based on search strategies in Medline and Cochrane Library from inception to October 2021 using the following terms: arthritis, psoriatic, psoriasis, gender, and sex. The panel discussed the results and proposed strategies by consensus.
Results
The scoping review identified broad themes: (1) clinical expression, (2) severity and patient-reported outcomes, (3) psychosocial impact, (4) access to treatments and propensity to treat, (5) comorbidities, and (6) treatment effect. The strategies are based on these broad themes.
Limitations
No risk of bias assessment was done due to the scoping nature of the review.
Conclusion
This review offers insights into gender differences in psoriasis, providing a foundation for improving clinical practice and patient outcomes.
... Palmoplantar skin lesions may occur exclusively in the palmoplantar region or, more regularly, concurrently with lesions in various anatomical regions in patients diagnosed with psoriasis vulgaris [3]. In a rather small percentage of psoriatic cases, hyperkeratotic scaly plaques may be complicated by developing sterile pustules [4,5]. ...
Background:
Palmoplantar psoriasis (PP) represents a localized type of disease. While controversy over its' classification exists, a hyperkeratotic type, a pustular type and palmoplantar pustulosis (PPP) have been recognized. PP management is regularly supported by biologic agents. Our study aimed to review and synthesize available data regarding the efficacy of approved biologics for PP and PPP.
Research design and methods:
A literature search was conducted in PubMed, CENTRAL, Scopus, and ClinicalTrilas.gov. Utilizing random-effects inverse-variance frequentist network meta-analyses (NMAs), we ranked interventions. The proportion of participants with cleared skin was the primary outcome. Fifty and 75% improvement in palmoplantar psoriasis area severity index (PPASI) were also explored (PPASI50, PPASI75).
Results:
In total, 15 randomized controlled trials (RCTs) exploring the efficacy of on-label adalimumab, bimekizumab, etanercept, guselkumab, infliximab, ixekizumab, secukinumab, and ustekinumab were included. Data for PP were synthesized. Every biologic agent examined, except from infliximab, outperformed placebo. On-label secukinumab exhibited the highest probability of inducing complete resolution. Ixekizumab and infliximab ranked best on inducing PPASI50 and PPASI75. Our review supports that guselkumab is effective for PPP.
Conclusions:
Secukinumab, ixekizumab and infliximab are effective for PP. Research is warranted to produce evidence about the efficacy of biologics in PP and PPP.
... Though it is found to be associated with psoriasis more than just by chance, it can occur in isolation too [ Figure 8] Cigarette smoking is strongly associated with palmoplantar pustulosis with up to 95% of patients with PPP having a positive history of smoking. 21 Apart from this, many other inciting factors like stress (43% patients), 40 nickel sensitivity (3% patients), 41 infections like tonsillitis, dental infections, the Koebner phenomenon, and hot and humid conditions have been mentioned. 32 A paradoxical flare of palmoplantar pustulosis with the anti TNFalpha agents is also noted. ...
Pustular psoriasis is a distinct subset of psoriasis that presents with involvement of the skin in the form of sterile pustules along with systemic manifestations. Though it has been conventionally grouped under the umbrella of psoriasis, recent research has shed light on its pathogenetic mechanisms associated with the IL-36 pathway, which is distinct from conventional psoriasis. Pustular psoriasis in itself is a heterogeneous entity consisting of various subtypes, including generalized, localized, acute, and chronic forms. There is confusion regarding its current classification as entities like deficiency of IL-36 antagonist (DITRA) which are closely related to pustular psoriasis both in their pathogenetic mechanism and its clinical manifestations, are not included under pustular psoriasis. Entities like palmoplantar pustulosis, which presents with similar clinical features but is pathogenetically distinct from other forms of pustular psoriasis, are included under this condition. Management of pustular psoriasis depends upon its severity; while some of the localized variants can be managed with topical therapy alone, the generalized variants like Von Zumbusch disease and impetigo herpetiformis may need intensive care unit admission and tailor-made treatment protocols. The advent of newer biologics and better insight into the pathogenesis of pustular psoriasis has opened the way for newer therapies, including tumor necrosis factor-alpha inhibitors, interleukin-1 inhibitors, interleukin-17 inhibitors, and granulocyte monocyte apheresis. It continues to be an enigma whether pustular psoriasis is actually a variant of psoriasis or an entirely different disease entity, though we feel that it is an entirely different disease process.
... P almoplantar pustulosis (PPP) is a chronic relapsing skin condition characterized by crops of sterile pustules together with redness and scaling on the palm and/or soles (1,2). The disease mainly affects women, and smoking is a well-established risk factor (3). Traditionally, PPP has often been classified as a subgroup of psoriasis, and between 14.2% and 61.3% of patients with PPP have co-occurring psoriasis vulgaris (4,5). Studies have reported that, in psoriasis populations, the PPP subgroup comprises between 3% and 10% (6)(7)(8)(9). ...
The aim of this study was to estimate the economic burden of palmoplantar pustulosis, a chronic relapsing skin condition commonly occurring in combination with psoriasis vulgaris. Using data from the Swedish National Patient Register and Swedish Prescribed Drug Register for 2015, the study estimated all-cause and palmoplantar pustulosis-specific healthcare resource use (inpatient stays, physician visits and drug use) for 14,715 patients with palmoplantar pustulosis, and compared these both with matched controls from the general population and with patients with psoriasis vulgaris (without palmoplantar pustulosis). Mean annual direct costs for a patient with palmoplantar pustulosis was higher compared with costs for the general population (3,000 vs 1,700 Euro, p < 0.001). Compared with psoriasis vulgaris, more patients with palmoplantar pustulosis had inpatient stays, but fewer had physician visits and psoriasis-related drugs; the overall costs were similar. Only a small fraction of the costs of physician visits and inpatient stays for patients with palmoplantar pustulosis were attributable to specific palmoplantar pustulosis problems, indicating a clear comorbidity burden in palmoplantar pustulosis.
... Brazilian Journal of Health Review, Curitiba, v. 5, n.6, p. 24233-24247, nov./dec., 2022 entre queratinócitos degenerados e achatados dentro da camada malpighiana superior da epiderme, formando uma macropústula subcórnea) e infiltrado predominantemente linfocitário perivascular na derme superior. A pústula espongiforme de Kogoj é um aspecto histológico característico da psoríase que é mais proeminente na variante pustulosa. ...
A psoríase pustular pode ser dividida em apresentações clínicas generalizadas e localizadas. A psoríase pustulosa generalizada (PPG) consiste em PPG aguda, também conhecida como psoríase pustulosa generalizada de von Zumbusch, e psoríase pustulosa anular generalizada, a psoríase é uma doença comum da pele caracterizada pelo desenvolvimento de placas eritematosas e descamativas na pele e um amplo espectro de apresentações clínicas. A apresentação mais comum da psoríase é a psoríase crônica em placas, que se manifesta como placas descamativas, vermelhas, bem definidas e inflamatórias na pele, a psoríase pustulosa é um subtipo menos comum de psoríase que se apresenta como uma erupção pustulosa aguda, subaguda ou crônica, a psoríase pustular afeta principalmente adultos, mas também pode ocorrer em crianças. As formas localizadas de psoríase pustulosa afetam principalmente as palmas das mãos, plantas dos pés ou extremidades dos dedos. Alguns casos de psoríase pustulosa generalizada ou localizada podem, na verdade, representar uma nova doença autoinflamatória genética baseada em mutações no antagonista do receptor da interleucina. A PPG ocorre mais comumente em adultos, mas também pode ocorrer em crianças. O reconhecimento de mutações no gene que codifica o antagonista do receptor de IL-36 ( IL36RN ) em alguns pacientes com PPG trouxe novos insights sobre a patogênese dessa condição.
... N1 number of patients who had a valid measurement value at the specified week, NAPSI nail psoriasis severity index, Q2W every 2 weeks. *P \ 0.05 versus ustekinumab and response to treatment are monitored using the palmoplantar PASI [44] and palmoplantar psoriasis physician's global assessment (PPPGA) [45][46][47]. ...
Psoriatic involvement in areas of the body such as nails, palms and soles (palmoplantar), and scalp is associated with dramatically negative effects on quality of life relative to involvement elsewhere in the body. Although numerous evidence-based studies demonstrate the efficacy of biologics for overall skin clearance in moderate-to-severe plaque psoriasis (including tumor necrosis factor α [TNFα] inhibitors and interleukin [IL]-17A, IL-12/IL-23, IL-23, IL-17F, and IL-17A/F inhibitors), large, randomized, placebo-controlled clinical studies of psoriasis with nail, palmoplantar, and scalp involvement are needed to better inform decision-making in clinical practice. Moreover, biologic failure caused by drug ineffectiveness is a common occurrence in patients who do not respond, lose response, or are intolerant to treatment. Brodalumab is a fully human IL-17 receptor A antagonist that demonstrates high rates of skin clearance among the latest generation of biologic therapies for treatment of moderate-to-severe psoriasis. This review summarizes current literature on the efficacy of brodalumab and other therapies in difficult-to-treat psoriasis including psoriasis in difficult-to-treat locations (such as psoriasis with nail, palmoplantar, or scalp involvement) and psoriasis in patients whose disease did not respond to other biologics.
... However, in a study done by Napolitano et al., female sex was significantly associated with psoriasis clinical type other than diffuse plaque psoriasis such as palmoplantar, guttate, arthro-pathic, pustular and localized plaque types (P=0.0001). Moreover, a palmoplantar pustulosis was found in a previous study to be predominant in female patients [17]. Furthermore, a number of studies have observed that generalized pustular psoriasis (GPP) is predominantly found in females in comparison to males [18][19][20]. ...
Introduction
Patients with psoriasis suffer from an inflammatory immune-mediated skin condition that impacts their quality of life severely. In our study, we aimed to analyze the gender differences in clinical characteristics in patients with psoriasis and to assess the quality of life in these patients.
Methods
This is a retrospective observational study that was conducted at King Abdulaziz Medical City (KAMC) in Jeddah, Saudi Arabia.
Results
A total of 139 patients with psoriasis met the inclusion criteria, with a mean ± SD age of 45.53 ± 15.3 years. No statistically significant gender differences were observed in terms of disease duration, BMI, clinical type and body surface area (BSA) (P=0.657, P=0.782, P=0.565, P=0.088, respectively). However, psoriasis caused a significant impairment in the quality of life in female patients compared to males (P=0.036).
Conclusion
This study reports the gender differences in clinical characteristics in patients with psoriasis and its impact on the quality of life. More researches with larger sample size and different populations are needed in order to identify more potential gender-specific variations.
... The FDA approved indications include iontophoretic delivery of fenatnyl, sumatriptan, lidocaine with epinephrine for post-operative pain, acute migraine and dermal analgesia respectively. In our study most cases were in the age group of 20-50 years which correlated with study by Khandpur S et al. [10] The mean age of onset was around 45 years which was like study by Brunasso AMG et al. [11] Males were affected with palmoplantar psoriasis relatively more in number than ~ 92 ~ www.dermatologypaper.com females which was in concurrence with study by Venkatesan A et al. [12] Both the palms and soles involvement showed higher frequency with 52.5% followed by soles alone (27.5%) and the palmar alone involvement with (17.5%) which was in accordance with Kumar improvement with methotrexate iontophoresis at 12 weeks which was lower than our study which could be attributed to lower frequency of iontophoresis [5] . ...
... Algunos autores consideran a esta entidad independiente de la psoriasis palmoplantar pustulosa; sin embargo, algunos otros sugieren que ambas comparten un mismo origen genético. [1][2][3] Por razones históricas, esta enfermedad ha recibido diferentes denominaciones: Pustulosis palmaris et plantaris, pustulosis palmoplantar persistente y psoriasis pustulosa de las extremidades, las cuales anteriormente se utilizaron como sinónimos. La controversia surgió a partir de la descripción de la denominada «Bacteride pustulosa de Andrews». ...
... 3 The group of diseases that involve bone joints is called pustulotic arthro-osteitis (PAO or Sonozaki syndrome). [4][5][6] PAO is said to occur in about 4.2%-45% of PPP cases 3,[7][8][9][10][11] and is frequently associated with smoking, dental lesions, and metal allergies. 12 Since the report by Sonozaki in 1981, 4 there have been few detailed reports on the clinical features of PAO, with the most recent report of a multicenter study in Japan with 165 subjects by Yamamoto et al. in 2020. ...
Pustulotic arthro-osteitis (PAO) is a major complication of palmoplantar pustulosis (PPP). In orthopedic surgery outpatient clinics, PPP patients with osteoarticular symptoms are seen frequently, but PAO's clinical features remain not well known. To determine Japanese patients’ clinical features and treatment status with PAO, we conducted a single-center retrospective epidemiologic survey. Clinical features, including gender, age, smoking habit, the onset pattern, interval between skin manifestation and osteoarticular symptoms, and the incidence of sternoclavicular joint lesions, axial and peripheral joint lesions, were examined. The association between physical status and image findings by X-ray, computed tomography, bone scintigraphy with Technetium⁹⁹, or magnetic resonance imaging was evaluated. The distribution pattern of peripheral joint lesions and the treatment status were evaluated.
We identified 51 patients, 10 men and 41 women, with PAO. The average age was 48 years and 59% were smokers. The frequency of onset patterns was skin-leading type (63%), simultaneous onset (18%), and osteoarticular leading type (16%). The average interval between skin involvement and osteoarticular involvement in skin-leading type was significantly longer than that in osteoarticular leading type (7.1 years vs. 2.0 years).
A sternoclavicular joint (SCJ) lesion was detected in 65% cases, and the physical findings of SCJ were significantly related to the image findings. Axial and peripheral joint lesions were detected in the same ratio (23 cases, 45%). In the peripheral joints, the finger joint was the most common (26%), followed by the shoulder joint (21%). Patients were treated with nonsteroidal anti-inflammatory drugs (76%), followed by conventional synthetic disease-modifying antirheumatic drugs (DMARDs) (29%) and biological DMARDs (9.8%). Tonsillectomy was performed in 11 cases. In conclusion, PAO more frequently involves SCJ in middle-aged women who smoke. Given that osteoarticular leading type was detected in 16% cases, seronegative oligoarthritis patients should be monitored for PPP, leading to a diagnosis of PAO.
... PPP tends to present later in life (mean age 48-69 yrs), and it is more common in women and in smokers. 6,7 The prevalence of PPP ranges from 0.01% in White populations to 0.12% in Japan, where it is associated with bacterial infections, including tonsillitis, sinusitis, or periodontal infections. 8,9 Tonsillectomy is reported to improve the symptoms of PPP. ...
Pustular psoriasis (PsO) is an uncommon variant of PsO that may present in a generalized or localized fashion with or without musculoskeletal or systemic inflammatory involvement. Generalized pustular PsO (GPP) presents as a widespread acute or subacute pustular eruption that may be familial and is often associated with severe flares and systemic inflammation. The palmoplantar pustulosis variant is localized to palms and soles, whereas acrodermatitis continua of Hallopeau is localized to the nail apparatus. Patients with pustular PsO may have overlapping plaque PsO and may develop psoriatic arthritis (PsA). Pustulosis is also a feature of both synovitis, acne, pustulosis, hyperostosis, osteomyelitis (SAPHO) syndrome and chronic nonbacterial osteomyelitis. At the 2020 Group for Research and Assessment of Psoriasis and Psoriatic Arthritis (GRAPPA) annual meeting, members were given an overview of the cutaneous features of pustular PsO, SAPHO, and recent insights into the genetics of GPP, leading to new targeted drug therapies and the development of validated endpoints.
Palmoplantar pustular psoriasis (PPPP), or palmoplantar pustulosis (PPP), is a type of psoriasis that affects the skin on the palms and soles. It is characterised by dermatosis and small sterile pustules and is considered a significant burden on patients' quality of life, as there is currently no gold standard treatment or cure. This network meta‐analysis (NMA) compares the efficacy and safety of biologic and non‐biologic medications for PPPP and PPP. Medline, Embase and the Cochrane Central Register of Controlled Trials (CENTRAL) were searched. The efficacy and safety of all medications were assessed through a frequentist NMA using a random‐effects model. Treatments were ranked using the net rank function, yielding P scores. Fourteen RCTs with 1056 participants were included. Guselkumab 100 mg was the most effective for improving PPPGA scores ( p = 0.72, RR = 1.31, CI: 0.31–5.57). Guselkumab 100 mg was ranked the highest for achieving PPPASI‐75 (RR = 5.4, CI: 1.26–23.2, p = 0.023). Oral cyclosporine 1 mg/kg/day was ranked the highest for PPPASI‐50 (RR = 2.10, CI: 0.65–6.82). Etretinate 1 mg/kg/day had the highest rate of adverse events (RR = 1.78, CI: 0.92–3.44). Secukinumab 300 mg was associated with the highest rate of serious adverse events (RR = 1.58, CI: 0.21–12.02). Based on the P‐scores from our NMA, guselkumab 100 mg was the most effective for PPPGA improvement, guselkumab 100 mg for PPPASI‐75, oral cyclosporine 1 mg/kg/day for PPPASI‐50, etretinate 1 mg/kg/day had the most adverse events, and secukinumab 300 mg was associated with the highest rate of serious adverse events.
Trial Registration
PROSPERO registration number: CRD42023460842
Background
Apremilast is a phosphodiesterase-4 (PDE-4) inhibitor approved by the US-Food and Drug Administration (US-FDA) for the management of adult patients with plaque psoriasis (who are candidates for phototherapy/systemic therapy), moderate-to-severely active psoriatic arthritis and Behcet’s disease associated with oral ulcers.
Purpose
This review aims to summarize the current evidence for the off-label use of apremilast in various dermatological disorders.
Methods
A comprehensive search of relevant literature was conducted using databases like PubMed, MEDLINE and Embase. Studies investigating the use of apremilast for dermatological conditions beyond its approved indications were included.
Results
The use of apremilast has been outlined in a number of reports for inflammatory dermatoses. Randomized controlled trials showed promise for atopic dermatitis, hidradenitis suppurativa and vitiligo. Open-label trials suggested potential benefit in cutaneous sarcoidosis, discoid lupus erythematosus, rosacea and lichen planus.
Conclusion
Apremilast can be considered a valid therapeutic option for a range of inflammatory dermatological conditions beyond its approved uses. Further, owing to lack of immunosuppression, apremilast delineates an added advantage when compared to the usual immunosuppressive drugs used by dermatologists. However, stronger evidence from well-designed clinical trials is needed to establish its role in these aforementioned disorders.
There is a complex interplay between psoriasis, a chronic immune-mediated skin condition, and its association with various co-occurring health concerns on a global scale. Psoriasis, which extends beyond just visible scaly red patches, reveals complex ties with conditions such as atherogenesis, insulin resistance, obesity, hypertension, dyslipidemia, metabolic syndrome, and diabetes mellitus. Psoriasis and diabetes have a significant correlation supported by epidemiological evidence. This chapter delves into the distinctive features of psoriasis and meticulously explores the intricate relationship it shares with diabetes. Uncovering potential connections and shared mechanisms, this exploration not only enriches our understanding of these conditions but also opens avenues for redefining diagnostic and treatment approaches to address both psoriasis and diabetes concurrently.
Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome and chronic nonbacterial osteomyelitis (CNO) are rare autoinflammatory/autoimmune conditions seen in adults and children. Although osteoarticular manifestations are the primary distinguishing features of SAPHO, over half of patients also have palmoplantar pustulosis (PPP). These and other associated disorders such as acne, inflammatory bowel disease, and hidradenitis suppurativa are characterized, at least in the early stages, by neutrophilic infiltration. The bone and skin manifestations exhibit both innate and adaptive immune responses and therefore share similar pathogenic molecules and overlapping treatment targets. At the Group for Research and Assessment for Psoriasis and Psoriatic Arthritis (GRAPPA) 2023 annual meeting, a 3-part presentation provided an overview of current efforts at establishing consensus on diagnosis/classification, treatment, and core outcome sets for SAPHO/CNO; an overview of PPP in SAPHO and as a standalone condition; and finally, an overview of the role of the neutrophil in these disorders.
There are limited data on acrodermatitis continua of Hallopeau (ACH), particularly among Asian populations. The primary aim was to evaluate the clinical features of ACH and treatment approaches in a sizeable multicentre Asian cohort. We analysed data from adult patients diagnosed with ACH. Of 65 patients with ACH, seven patients had ACH with GPP. Females were more frequently affected in both conditions. Five (71.4%) developed GPP 5–33 years after ACH onset, while two (28.6%) developed GPP concurrently with ACH. The onset age for ACH with GPP (27.9 ± 13.6 years) was earlier than that of isolated ACH (39.8 ± 17.3 years). Metabolic comorbidities were common. ACH exhibited a chronic persistent course. Among systemic non‐biologics, acitretin was the most frequently prescribed, followed by ciclosporin and methotrexate. Acitretin and ciclosporin demonstrated similar marked response rates, which surpassed that of methotrexate. Regarding biologics, a marked response was more commonly observed with interleukin‐17 inhibitors than with tumour necrosis factor inhibitors. Females are predominant in both conditions. The onset age for ACH among Asian patients is earlier (late 30s) than that for Caucasian patients (late 40s). Interleukin‐17 inhibitors may be more effective than tumour necrosis factor inhibitors in managing ACH.
Background
Data on the treatment of palmoplantar psoriasis (PP) are very limited as these patients are often excluded from clinical trials. Moreover, this form of psoriasis is often resistant to treatment, making its clinical management complex.
Methods
Primary endpoint was to evaluate the clinical and demographic characteristics and the drug survival of both biological and non‐biological drugs in a population affected by PP. Secondary endpoint was to highlight any differences between the hyperkeratotic and pustular variant. We analyzed data from 233 psoriasis patients with palmoplantar involvement, with or without chronic plaque psoriasis. We performed a drug‐survival analysis with the aid of Kaplan‐Meier survival and a multivariate analysis to highlight the influence of certain variables on treatment persistence using a Cox regression model.
Results
The drug‐survival analysis revealed that biologic drugs compared to non‐biologic drugs are associated with a higher persistence in treatment (59.73 vs. 43.56%); in particular, anti‐IL23 drugs were found to be the drugs with the best drug‐survival overall (67.94% of patients at 60 months are still on these drugs). Furthermore, our multivariate analysis shows that when compared with biological drugs, non‐biological drugs are associated with an increased risk of treatment discontinuation (HR = 1.95 [95% CI: 1.41–2.68], P = 0.001).
Conclusions
Our study confirms the difficulty of treating PP and shows that biologic drugs are associated with longer persistence in treatment than non‐biologics in both PP's variants, not because of their higher effectiveness but because of their better safety profile.
Background and objective:
Pustular psoriasis is a chronic and recurrent autoimmune disease, although little is known about the disease burden of pustular psoriasis in China. We analyzed the characteristics and disease burdens of patients from Beijing who had generalized pustular psoriasis (GPP) or palmoplantar pustulosis (PPP).
Methods:
This multicenter retrospective cohort study used a regional electronic health database that covered 30 public hospitals in Beijing. From June 2016 to June 2021, all patients with a diagnosis of GPP, PPP, or psoriasis vulgaris (PV) were identified by International Statistical Classification of Diseases and Related Health Problems, 10th Revision codes. The GPP and PPP cohorts were separately matched with patients with PV in a 3:1 ratio for comparisons. Demographic data, clinical characteristics, healthcare resource utilization, and costs were collected. Descriptive and comparative analyses were used to compare the cohorts.
Results:
There were 744 patients with GPP (46.8% men; age 42.14 ± 21.47 years) and 4808 patients with PPP (35.5% men; age 51.65 ± 16.12 years); 14.5% of patients with GPP had concomitant PV and 7.5% of patients with PPP had concomitant PV. Relative to matched patients with PV, patients with GPP had a higher prevalence of erythrodermic psoriasis (5.9% vs 0.4%, p < 0.0001), psoriatic arthritis (3.1% vs 1.5%, p = 0.007), and organ failure (1.1% vs 0.2%, p = 0.002). Relative to matched patients with PV, patients with PPP had a higher prevalence of cerebrovascular disease (4.7% vs 1.2%, p < 0.0001), thyroid dysfunction (3.9% vs 3.3%, p = 0.035), and type 2 diabetes mellitus (6.8% vs 5.9%, p = 0.030). More patients with GPP than patients with PV received systemic non-biological agents (27.9% vs 3.3%, p < 0.0001) and biologic agents (4.8% vs 2.0%, p = 0.010). More patients with PPP than patients with PV received topical agents (50.9% vs 34.7%, p < 0.0001) and systemic non-biological agents (17.8% vs 2.7%, p < 0.0001). More patients with GPP than patients with PV required inpatient hospitalization (22.0% vs 7.8%, p < 0.0001). Hospitalization stay was longer in patients with GPP than patients with PV (11.72 ± 0.45 vs 10.38 ± 0.45 days, p = 0.022). More patients with PPP than patients with PV had emergency visits (16.3% vs 12.8%, p < 0.0001). The GPP and PPP cohorts and their matched PV cohorts had no significant differences in costs. However, patients with PPP had lower outpatient costs than patients with PV (368.20 ± 8.19 vs 445.38 ± 5.90 Chinese Yuan per patient per month, p < 0.0001).
Conclusions:
Patients from Beijing with GPP and PPP had higher disease burdens than matched PV cohorts, including the prevalence of comorbidities, healthcare resource utilization, and medication burden. However, the economic burden of pustular psoriasis was similar to that of PV. Practical and specific therapies are needed to reduce the burdens of pustular psoriasis.
Introduction:
Palmoplantar pustulosis (PPP) is a chronic inflammatory skin disease belonging to the localized form of pustular psoriasis. It is characterized by sterile pustule formation in palms and soles and a recurrent disease course. Although we have many treatments for PPP, there is no authoritative guidance.
Areas covered:
A thorough search of PubMed was conducted to identify studies in PPP from 1973 onwards, with additional references to specific articles. Any treatment methods were outcomes of interest, including topical treatment, systemic treatment, biologics, other targeted treatments, phototherapy, and tonsillectomy.
Expert opinion:
Topical corticosteroids are suggested as first-line therapy. Oral acitretin has become the most applied systemic retinoid recommended in PPP without joint involvement. For patients with arthritis, immunosuppressants like cyclosporin A and methotrexate are more recommended. UVA1, NB-UVB, and 308-nm excimer laser are effective phototherapy options. The combinations of topical or systemic agents and phototherapy may enhance the efficacy, particularly in recalcitrant cases. Secukinumab, ustekinumab, and apremilast are the most investigated targeted therapies. However, heterogeneous reported outcomes in clinical trials provided low-to-moderate quality evidence of their efficacy. Future studies are required to address these evidence gaps. We suggest managing PPP based on the acute phase, maintenance phase, and comorbidities.
Palmoplantar pustulosis (PPP) is a variant of pustular psoriasis involving the palms and soles. The severity of PPP is usually evaluated using the Palmoplantar Pustulosis Area and Severity Index (PPPASI). Among the components of the PPPASI, the area of the involved lesion is evaluated differently by raters, who generally make a rough estimate using the eye and not through a specific calculation. To overcome inconsistent evaluation of the area subscore of PPPASI by human raters, we developed and validated deep-learning-based algorithms to enable automated and reliable assessment of the area involved in PPP to provide clinical advantages. In this study, we developed a dataset of 611 images of the palms and soles of 153 patients with PPP. We evaluated the area of the lesion by dividing the number of pixels in the area involved in PPP by the number of pixels in the area of the palms or soles. Using attention U-net, we developed two convolutional neural network (CNN) models that can evaluate the percentage of the affected area (%) and subsequently assign a score ranging from 0 to 6. The area subscore of PPPASI evaluated by the deep-learning algorithm was same or differed by 1-point from the subscore of ground truth in 98.8% of the images. The intraclass correlation coefficient between the CNN and ground truth was 0.879, indicating good agreement. The accuracy and mean absolute error of the model were 66.7% and 0.344, respectively. In a Bland-Altman plot, most of the differences in the percentage of the affected area lay between the 95% confidence interval with a mean difference of 0 and a standard deviation of 0.2. The deep-learning algorithm can provide several clinical advantages by objectively evaluating the components of the PPPASI without concern for disagreement between clinicians. The algorithms further enable cumulative clinical data acquisition related to PPP severity.
Palmoplantar pustular psoriasis (PPPP) and non-pustular palmoplantar psoriasis (NPPP) are localized, debilitating forms of psoriasis. The inflammatory circuits involved in PPPP and NPPP are not well understood. To compare the cellular and immunological features that differentiate PPPP and NPPP, skin biopsies were collected from a total of 30 participants with PPPP, NPPP, and psoriasis vulgaris (PV) and from 10 healthy participants. A subset consented to a second biopsy after 3 weeks off medication. Histologic staining of lesional and non-lesional skin showed higher neutrophil counts in PPPP compared with NPPP and PV, and higher CD8⁺ T-cell counts in NPPP. RNA sequencing and transcriptional analysis of skin biopsies showed enhanced IFN-γ pathway activation in NPPP lesions, but stronger signatures of IL-17 pathway and neutrophil-related genes (e.g. IL36A) in PPPP lesional skin. Serum analysis on the Olink platform detected higher concentrations of T helper (Th) type 1, IFN-γ inducible chemokines in NPPP and higher neutrophil-associated cytokines in PPPP. Taken together, this evidence suggests more pronounced Th1-mediated inflammation in NPPP compared to PV and PPPP, and stronger neutrophil-associated activity in PPPP compared to NPPP and PV. These data support targeting inflammatory pathways associated with neutrophilic inflammation (e.g. IL-36 signaling) for therapeutic development in PPPP.
Dear Editor, Pustular psoriasis is a group of inflammatory skin conditions characterized by clinically visible sterile pustules. It has been considered as a form of psoriasis vulgaris, but they are phenotypically different, respond differently to treatments and are genetically distinct. Variations in IL36RN, CARD14, APIS3, MPO and SERPINA3 genes have been linked to generalized pustular psoriasis.¹,² The European Rare and Severe Psoriasis Expert Network (ERASPEN) recently presented a consensus classification of clinical phenotypes of pustular psoriasis.³ There are limited data on the differences between pustular and plaque psoriasis. Therefore, we aimed to compare the demographic characteristics, comorbidities and prescriptions between these clinical variants in clinical practice.
We used data from the BIOBADADERM registry, a previously described prospective multicentre cohort registry of patients with psoriasis treated with systemic drugs in Spain intended to detect adverse events related to systemic therapy.⁴ Participants enter the cohort when they start a therapy that they have never used before. This study contains analysis of the data extracted from BIOBADADERM from October 2008 to December 2021. Therapy in the first 5 years of disease was described in a subset of patients with disease onset close to cohort entry. Plaque psoriasis (PP) was used as the reference group for all comparisons.
Importance:
Palmoplantar pustulosis (PPP) has been reported to be accompanied by systemic conditions. However, the risks of comorbidities in patients with PPP have rarely been evaluated.
Objective:
To assess the risks of comorbidities in patients with PPP compared with patients with psoriasis vulgaris or pompholyx.
Design, setting, and participants:
This nationwide population-based cross-sectional study used data from the Korean National Health Insurance database and the National Health Screening Program collected from January 1, 2010, to December 31, 2019. Data were analyzed from July 1, 2020, to October 31, 2021. Korean patients diagnosed with PPP, psoriasis vulgaris, or pompholyx who visited a dermatologist between January 1, 2010, and December 31, 2019, were enrolled.
Exposures:
Presence of PPP.
Main outcomes and measures:
The risks of comorbidities among patients with PPP vs patients with psoriasis vulgaris or pompholyx were evaluated using a multivariable logistic regression model.
Results:
A total of 37 399 patients with PPP (mean [SD] age, 48.98 [17.20] years; 51.7% female), 332 279 patients with psoriasis vulgaris (mean [SD] age, 47.29 [18.34] years; 58.7% male), and 365 415 patients with pompholyx (mean [SD] age, 40.92 [17.63] years; 57.4% female) were included in the analyses. Compared with patients with pompholyx, those with PPP had significantly higher risks of developing psoriasis vulgaris (adjusted odds ratio [aOR], 72.96; 95% CI, 68.19-78.05; P < .001), psoriatic arthritis (aOR, 8.06; 95% CI, 6.55-9.92; P < .001), ankylosing spondylitis (aOR, 1.91; 95% CI, 1.61-2.27; P < .001), type 1 diabetes (aOR, 1.33; 95% CI, 1.16-1.52; P < .001), type 2 diabetes (aOR, 1.33; 95% CI, 1.29-1.38; P < .001), Graves disease (aOR, 1.25; 95% CI, 1.11-1.42; P < .001), Crohn disease (aOR, 1.63; 95% CI, 1.11-2.40; P = .01), and vitiligo (aOR, 1.87; 95% CI, 1.65-2.12; P < .001) after adjusting for demographic covariates. The risks of ankylosing spondylitis (aOR, 1.37; 95% CI, 1.16-1.62; P < .001) and Graves disease (aOR, 1.40; 95% CI, 1.23-1.58; P < .001) were significantly higher among patients with PPP vs psoriasis vulgaris. However, the risks of psoriatic arthritis (aOR, 0.54; 95% CI, 0.47-0.63; P < .001), systemic lupus erythematosus (aOR, 0.67; 95% CI, 0.46-0.97; P = .04), Sjögren syndrome (aOR, 0.70; 95% CI, 0.50-0.96; P = .03), systemic sclerosis (aOR, 0.29; 95% CI, 0.11-0.77; P = .01), vitiligo (aOR, 0.53; 95% CI, 0.47-0.60; P < .001), and alopecia areata (aOR, 0.88; 95% CI, 0.81-0.95; P = .001) were significantly lower among those with PPP vs psoriasis vulgaris.
Conclusions and relevance:
The results of this cross-sectional study suggest that patients with PPP have an overlapping comorbidity profile with patients with psoriasis vulgaris but not patients with pompholyx. However, the risks of comorbidities among patients with PPP may be substantially different from those among patients with psoriasis vulgaris.
Background:
Palmoplantar pustulosis (PPP) and palmoplantar pustular psoriasis (PPPP) are chronic inflammatory skin conditions characterized by eruptions of sterile pustules on the palms and/or soles. Biologic use has been associated with PPP and PPPP development in the literature.
Objectives:
To identify PPP and PPPP associated with biologics and summarize reported treatments and outcomes.
Methods:
We systematically searched in MEDLINE and Embase for articles that reported PPP or PPPP during biologic treatment. After a full-text review, 53 studies were included for analysis.
Results:
We identified 155 patients with PPP/PPPP onset during biologic treatment, with a mean age of 44.1 years and a female preponderance (71.6%). The most frequently reported biologics were adalimumab (43.9%) and infliximab (33.3%). IL-17 inhibitors, secukinumab (7.6%) and brodalumab (1.5%), were reported only in association with PPPP. Overall, 58.8% of patients had complete remission (CR) in 3.6 months and 23.5% had partial remission (PR) in 3.7 months. The most common treatments that led to CR were topical corticosteroids (n = 16) and biologic switching (n = 8).
Conclusions:
Clinicians should anticipate PPP or PPPP as potential drug reactions to biologics such as adalimumab and infliximab. Large-scale studies are required to confirm our findings and further explore the pathogenesis for biologic-associated PPP and PPPP.
Importance
Palmoplantar pustulosis (PPP) is a is a chronic, orphan disease with limited epidemiological data.
Objective
To describe the clinical characteristics, treatments, longitudinal disease course, and health care utilization in adults with PPP across the US.
Design, Setting, and Participants
This retrospective, longitudinal case series from 20 academic dermatology practices in the US included a consecutive sample of 197 adults who met the European Rare and Severe Psoriasis Expert Network consensus definition for PPP between January 1, 2007, and December 31, 2018. Data analysis was performed June 2020 to December 2020.
Main Outcomes and Measures
The primary outcome was to describe the patient characteristics, associated medical comorbidities, treatment patterns, complications, and PPP-specific health care utilization.
Results
Of 197 patients, 145 (73.6%) were female, and the mean (SD) age at presentation was 53.0 (12.6) years, with a mean (SD) follow-up time of 22.1 (28.0) months. On initial presentation, 95 (48.2%) patients reported skin pain, and 39 (19.8%) reported difficulty using hands and/or feet. Seventy patients (35.5%) were treated with systemic treatments, and use of more than 20 different systemic therapies was reported. In patients with at least 6 months of follow-up (n = 128), a median (IQR) of 3.7 (4-10) dermatology visits per year were reported; 24 (18.8%) patients had 5 or more visits during the study period.
Conclusions and Relevance
In this case series, PPP was associated with persistent symptoms, continued health care utilization, and a lack of consensus regarding effective treatments, emphasizing the unmet medical need in this population. Additional research is necessary to understand treatment response in these patients.
Guselkumab is a human IgG1λ monoclonal antibody that has been approved for treatment of multiple immunological diseases including palmoplantar pustulosis (PPP) in Japan. The efficacy of guselkumab in reducing disease severity as compared with placebo has been demonstrated in phase 2 and 3 clinical studies. In some patients assigned to the placebo treatment, worsening of PPP Area and Severity Index (PPPASI) score was noted. Most of these patients were smokers, raising a possibility of an association of smoking with the disease progression. To understand the clinical implications of guselkumab dose, baseline disease severity and smoking on the treatment effect and describe the longitudinal relationship between guselkumab exposure and the PPPASI score, a PK/PD modeling analysis was conducted using the pooled data from one phase 2 and one phase 3 study. Data from 207 Japanese patients (77% women and 60% smokers) with a median PPPASI score of 24.6 were included in the analysis. The observed treatment efficacy (the PPPASI score reduction) appeared to be similar at the current approved dose (100) mg and the higher dose (200 mg). Greater extent of the PPPASI score reduction (in absolute points) is expected in patients with higher baseline PPPASI score (severe disease). However, the higher baseline did not translate to larger magnitude of the change from baseline (in percentage) in the PPPASI score. Incorporating a linear disease progression effect in the model significantly decreased the NONMEM objective function value (p<0.001). Smoking status appeared tobe related to disease worsening in some patients, but the covariate did not reach statistical significance in the model. This article is protected by copyright. All rights reserved
Palmoplantar pustulosis (PPP) is a chronic inflammatory condition where crops of sterile pustules with erythematous keratotic lesions causing bleeding and pain appear on the palms and soles. Recently, the European Rare and Severe Expert Network considered PPP as a variant of pustular psoriasis with or without psoriasis vulgaris.
The prevalence of PPP varies from 0.050 to 0.12%. PPP occurs more frequently in women and the highest prevalence occurred between the ages of 50 and 69 years. Nail psoriasis seems to be frequent in PPP, ranging from 30 to 76%, and psoriatic arthritis in 8.6 to 26% of PPP patients.
Synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome and pustulotic arthro-osteitis are considered PPP-associated disorders. PPP has been reported with other co-morbidities such as psychiatric disorders, thyroid-associated disease, altered calcium homeostasis, gluten sensitivity diabetes, obesity, and dyslipidemia, but larger studies are required to prove such associations. Environmental exacerbating factors might contribute to the onset or worsening of PPP such as cigarette smoking, stress, focal infections, metal allergies, and drug intake. Genetic predisposition plays an important role in PPP.
In PPP, both the innate and the adaptive immune systems are activated. The acrosyringeal expression of IL-17 has been demonstrated, indicating that the eccrine sweat gland is an active component of the skin barrier and an immune-competent structure. Increased levels of several inflammatory molecules, including IL-8, IL-1α, IL-1β, IL-17A, IL-17C, IL-17D, IL-17F, IL-22, IL-23A, and IL-23 receptor, have been detected in PPP biopsies. Increased serum levels of TNF-α, IL-17, IL-22, and IFN-γ have been detected in patients with PPP in comparison to healthy subjects, suggesting a similar inflammatory pattern to psoriasis vulgaris. Oral and tonsillar infections serve as trigger factors for PPP.
Long-term therapy is required for many patients, but high-quality data are limited, contributing to uncertainty about the ideal approach to treatment.
Background
Placebo-controlled studies have reported the efficacy of apremilast in the management of palmoplantar psoriasis but studies comparing efficacy with a conventional agent are lacking.Objective
The objective of this article was to compare the efficacy and safety of apremilast and methotrexate in patients with palmoplantar psoriasis.Methods
In this prospective, randomized, active-controlled, observer-blinded clinical trial, conducted at a psoriasis clinic of a tertiary care institute in India from 1 July, 2019 to 1 June, 2020, 84 patients with palmoplantar psoriasis were randomized (1:1) to receive either methotrexate (0.4 mg/kg/week orally) or apremilast (30 mg twice daily). The treatment protocol was continued for 16 weeks or until achieving a ≥ 75% improvement in the Modified Palmoplantar Psoriasis Area and Severity Index (m-PPPASI 75), whichever was earlier. Changes in m-PPPASI and Dermatology Life Quality Index scores from baseline, the proportion of patients achieving m-PPPASI 75, and adverse events were assessed.ResultsEighty-four patients were included (76 palmoplantar psoriasis and 8 palmoplantar pustulosis). The mean age (standard deviation) was 44.5 (12.9) years and 53 (63%) were women. The m-PPPASI score [median (interquartile range)] after 16 weeks of treatment showed a significant improvement from baseline in both apremilast [− 6.3 (10.9), p < 0.001] and methotrexate groups [− 8.5 (9.9), p < 0.001]. The estimated median difference between the groups was − 1.2 (p = 0.39, 95% confidence interval − 4.2 to 2.1). At 16 weeks, m-PPPASI 75 was achieved by 14/42 (33%) and 17/42 (41%) patients in the apremilast and methotrexate groups, respectively (p = 0.49). A significant reduction in the Dermatology Life Quality Index score [median (interquartile range)] was observed in both groups [apremilast: − 3.0 (6.0), p < 0.001; methotrexate: − 3.0 (6.3), p < 0.001] with an estimated median difference of 0.0 (p = 0.99, 95% confidence interval − 1.0 to 2.0). The proportion of patients experiencing adverse events was comparable (p = 0.49).Conclusions
Apremilast showed a comparable efficacy and safety profile to methotrexate in the management of palmoplantar psoriasis.Clinical Trial RegistrationCTRI/2019/06/019830, date of registration: 24 June, 2019; trial registered prospectively.
Hand and foot psoriasis is a disabling condition associated with significant quality-of-life issues. It is characterized by hyperkeratosis and/or the development of recurrent crops of sterile pustules with associated erythema, fissuring, and scaling symmetrically localized on palm and soles. Systemic conventional therapies include retinoids, psoralen-UVA (PUVA), methotrexate, and cyclosporine. So far, only limited evidence supports the use of TNF-alfa blockers. Because there are reports of paradoxical induction of pustular psoriasis following the use or withdrawal of infliximab as well as other TNF inhibitors, the use of these agents in palmoplantar psoriasis has been cautioned. The authors describe the clinical characteristics and evolution of 4 adult patients with severe palmoplantar psoriasis who were treated successfully with infliximab. Patient data is available for a minimum of 10 months and as many as 16. One of them with concomitant HCV infection showed no increased viral replication or progression of liver disease for a follow-up of 10 months; afterwards infliximab was stopped because of an infusion-related urticaria. All other patients displayed a good clinical response (> or =PPPASI 50) and were still receiving this regimen at last observation. This report provides preliminary evidence to support a cautious use of infliximab in patients with palmoplantar psoriasis.
Although palmoplantar psoriasis can be severely disabling, there are very few large clinico-epidemiological studies on this condition. Our purpose was to study the morphology and pattern of lesions in Indian patients with palmoplantar psoriasis and to elucidate the role of occupation in the incidence/localization of these lesions. All patients attending our Psoriasis Clinic from 1993 to 2000 were screened for palmoplantar lesions and their demographic characteristics, occupation and the exact localization of the lesions were noted. Out of 3,065 patients screened, 532 had palm and/or sole involvement. Plantar lesions were seen in 91.9% and palmar lesions in 55.6% of these patients. Four distinct patterns of lesion localization were noted on the palms and 5 patterns on the soles. Almost half of the men involved in regular manual labor had palmar lesions restricted to areas exposed to pressure, whereas only a quarter of other men had this type of lesion pattern. All patients with unilateral palmar lesions had them on their dominant hand and these patients were involved in regular manual labor. In our patients, the prevalence of plantar lesions was much higher than that of palmar lesions. The possible role of occupational trauma in lesion localization in Indian patients with palmoplantar psoriasis is discussed.
Ninety-five percent of patients with palmoplantar pustulosis are smokers at onset of the disease. The aim of this study was to determine whether these patients have serum antibodies to nicotinic acetylcholine receptors (nAChR ab) and if their sera induce a specific immunofluorescence in normal palmar skin. Sera from 45 patients with palmoplantar pustulosis and 23 patients with chronic hand eczema were analysed for muscle nAChR ab, and immunofluorescence was performed on healthy palmar skin. Forty-two percent of the patients with palmoplantar pustulosis but none of the eczema patients had raised levels of nAChR ab. Immunofluorescence showed staining on endothelial cells in the papillary dermis in 47% of all sera from patients with palmoplantar pustulosis and in those with nAChR ab in 68%. On palmar skin from smokers there was also a staining of the sweat duct. Sera from patients with chronic hand eczema were negative. Our findings indicate that palmoplantar pustulosis is an autoimmune disease, possibly induced by smoking.
Palmoplantar pustulosis is characterized by pustule formation in the acrosyringium. Nearly 50% of palmoplantar pustulosis sera produce immunofluorescence of the palmar papillary endothelium from healthy subjects, but also of the endothelium of normal parathyroid gland. With a case-control design the levels of calcium and parathyroid hormone in serum were measured in 60 women with palmoplantar pustulosis and 154 randomly selected population-based control women. One-third of the controls had been smokers, whereas 95% of the cases were or had been smokers. Mean age-adjusted serum calcium was increased in the patients compared with the controls (2.43 vs 2.36 mmol/l; p<0.0001), whereas the parathyroid hormone concentration was suppressed (23.2 vs 31.1 ng/l; p<0.0001). The plasma levels of parathyroid hormone-related protein were normal in patients but there was a strong expression of this protein in the acrosyringium both in palmoplantar pustulosis and control skin. As even a marginal elevation of serum calcium is associated with an increased risk for diabetes, cardiovascular disease and psychiatric disease, we analysed the risk for these disorders in palmoplantar pustulosis patients compared with that in the control group. Both diabetes mellitus and psychiatric disorders were associated with palmoplantar pustulosis with an odds ratio of 8.7 (95% CI 3.3-22.8) and 5.6 (95% CI 2.2-14.4), respectively. Palmoplantar pustulosis is a complex disease with an increased risk for several non-dermatological disorders. The role of the mildly increased serum calcium for the high risk for diabetes and depression deserves to be studied.
We report a 13-year-old girl with severe pustular psoriasis who had an excellent response to treatment with adalimumab after failure with methotrexate, acitretin, cyclosporin, phototherapy, and biologic drugs including etanercept and infliximab.
We report a 10-year-old boy presenting with palmoplantar pustular psoriasis, resistant to topical and systemic treatments, who was successfully treated with subcutaneous etanercept (0.4 mg/kg) twice a week for 1 month. Maintenance therapy was extended for 18 months in combination with near ultraviolet light therapy without any adverse effect. Etanercept may be a safe and effective alternative for severe palmoplantar pustular psoriasis in children.
Palmoplantar psoriasis is a difficult to treat variant of plaque psoriasis.
To study the safety and efficacy of infliximab in non-pustular palmoplantar psoriasis.
Patients with non-pustular palmoplantar psoriasis affecting at least 10% of their palms and soles and with a modified palmoplantar psoriasis area and severity index (m-PPPASI) of at least eight were recruited. Patients were randomized (1:1) to receive infliximab 5 mg/kg or placebo at weeks 0, 2 and 6. Patients initially randomized to placebo received infliximab at weeks 14, 16 and 20 whereas patients randomized to infliximab received additional infliximab infusions every 8 weeks until week 22.
Twenty four (24) patients were randomized in this study. At week 14, 33.3% and 66.7% of patients treated with infliximab achieved m-PPPASI 75 and m-PPPASI 50 respectively compared to 8.3% for both m-PPPASI 75 (P = 0.317) and m-PPPASI 50 (P = 0.009) for patients randomized to placebo. A reduction of 50.3% in the mean surface area of palms and soles affected with psoriasis was seen at week 14 in patients randomized to infliximab as compared to an increase of 14.9% in patients randomized to placebo (P = 0.009).
This pilot study did not reach its primary endpoint of m-PPPASI 75 at week 14. However, infliximab was observed to be more efficacious than placebo in improving PPSA and with respect to the percentage of patients reaching m-PPPASI 50 at week 14. Larger and longer term studies are needed for severe patients to better assess the efficacy of infliximab in palmoplantar psoriasis.
Leprosy is an infectious disease characterized by a wide spectrum of clinical manifestations, ranging from tuberculoid to lepromatous disease with immunologically unstable borderline forms in between. In clinical practice cases often do not conform to a classical textbook description, which may lead to misdiagnosis if not properly investigated. A 22-year-old patient presented to us with erythematous plaques localized to the face. Slit skin smear for Mycobacterium leprae was positive from lesional as well as non-lesional skin. A biopsy from a plaque showed diffuse atrophy of the epidermis with a subepidermal cell free zone (grenz zone). The cellular infiltrate was composed of foamy macrophages admixed with lymphocytes in the dermis. Fite-Faraco staining revealed clumps of acid-fast bacilli within the macrophages. Based on the skin smear and histopathology findings, a diagnosis of sub-polar lepromatous leprosy was made and the patient was started on multidrug therapy. The exact pathogenesis of localized multibacillary disease is not known. Our case highlights the importance of skin smear and biopsy in all suspected cases of Hansen disease. We conclude that routine skin smear in all new leprosy cases is mandatory to differentiate localized multibacillary cases from paucibacillary cases for the purpose of accurate categorization and treatment.
Pustulosis palmoplantaris (PPP) is a chronic inflammatory skin disease with a considerable impairment of quality of life and is characterized by sterile pustules and red, scaly skin on the palms and soles. The position of PPP in relation to psoriasis is unclear. Some authors regard PPP as a distinct disease entity, whereas others categorize PPP as a manifestation of psoriasis. Related to this discussion is the question on the treatment of PPP. Should the treatment of PPP follow the guidelines for psoriasis or is it a disease that has to be treated in a different way? The purpose of this editorial is to review the differences between PPP and psoriasis and to understand these differences with respect to the pathogenesis and treatment.
A wide variety of scoring systems have been proposed to assess severity of psoriasis. Given its importance as a health issue both for patients and health care systems, it is critically important to evaluate the validity and reliability of existing outcome measures.
The objective of this systematic review was to assess the extent of validation including the validity, reliability, sensitivity to change and ease of use of available outcome measures for psoriasis.
We conducted a systematic review of all clinical studies (prospective and retrospective) investigating the severity of psoriasis patients and published between January 1980 and June 2009. The following methodological validation and quality criteria were recorded systematically: construct validity, content validity and internal consistency, intra-observer variation and inter-observer variation, sensitivity to change and acceptability/ease of use assessed as time required to perform measurement.
Based on methodological validation and quality criteria, six clinical severity scores were selected and analysed (PASI, BSA, PGA, LS-PGA, SPI and SAPASI scores). We did not find substantial evidence of construct validity for any of the psoriasis clinical severity scores. Content validity was studied by considering the PASI score as gold standard. The relative content validity was good for the LS-PGA, PGA, and SPI scores, which correlated strongly with the PASI score. The SAPASI and PASI scores showed moderate correlation. Internal consistency was good for the PASI and LS-PGA scores. The PASI, BSA, PGA and LS-PGA scores displayed limited intra-observer variation. The inter-observer variation was low for LS-PGA (ICC < 0.5) and SAPASI, moderate for PASI, SPI and PGA and high for BSA (ICC > 0.8). The PASI score and the SAPASI displayed moderate sensitivity to change.
Based on this systematic review, it appears that none of the severity scores used for psoriasis meets all of the validation criteria required for an ideal score. However, we can conclude that the PASI score is the most extensively studied psoriasis clinical severity score and the most thoroughly validated according to methodological validation criteria. Despite certain limitations, use of the PASI score can be recommended for scientific evaluation of the clinical severity of psoriasis.
Treatment options remain unsatisfactory for patients with palmoplantar psoriasis (PP) and palmoplantar pustular psoriasis (PPP). Aim To evaluate the therapeutic responses of PP and PPP patients that were treated in our psoriasis polyclinic between 2003 and 2007.
This retrospective study comprised PP (n = 62) and PPP (n = 52) patients. Treatments were individualized according to patient compliance and associating systemic diseases. The effect of systemic treatments was grouped as follows: 'no improvement': patients unresponsive for the present treatment; 'partial improvement': < 50% decrease in severity or affected area; 'moderate improvement': 50-75% decrease in severity or affected area, and 'marked improvement': > 75% decrease of the disease compared to baseline.
In the PP group, 17 of 62 patients showed marked improvement to topical agents, while the remaining patients required systemic agents including oral retinoids (n = 24), local psoralen plus ultraviolet A (PUVA; n = 12), methotrexate (n = 9) and cyclosporine (n = 2). Marked improvement was achieved in 53%, 45%, 47% and 100%, respectively. In these patients, two (n = 10), three (n = 5), or four (n = 5) systemic agents were used alternately. In the PPP group, 18 of 52 patients achieved marked improvement by topical agents. Patients that required systemic agents were treated with colchicum (n = 19), local PUVA (n = 8), methotrexate (n = 4), oral retinoids (n = 3) and cyclosporine (n = 2). These treatments achieved a marked improvement in 60%, 33%, 57%, 83%, and 50% of the patients, respectively. In the course of the disease, 18 patients required two and 3 patients required three systemic agents alternately.
Although the success rates appeared to be high, the high number of patients who required multiple systemic agents emphasized the fact that localized forms of psoriasis were resistant to therapy.
Palmoplantar psoriasis is associated with significant quality-of-life issues. Its epidemiology and phenotypical expression remain ill defined.
We reviewed the literature and our clinical experience and developed a new quality-of-life assessment tool.
We conducted a retrospective review of 150 patients with palmoplantar psoriasis.
In all, 78 (52%) patients displayed predominantly hyperkeratotic palmoplantar lesions, 24 (16%) pustular, 18 (12%) combination, and 30 (20%) had an indeterminate phenotype. In 27 (18%) patients, lesions were confined to the palms and soles. A new quality-of-life index was constructed to characterize disease severity. In all, 27 (18%) had mild, 72 (48%) moderate, and 51 (34%) severe disease involvement. Palmoplantar disease severity appeared independent from the degree of body surface area involvement.
This was a retrospective review. The quality-of-life index remains to be statistically verified in prospective clinical studies.
Defining morphologic subtypes together with the use of a specific quality-of-life assessment tool in patients with palmoplantar psoriasis will improve our understanding and treatment of this recalcitrant form of psoriasis.
Palmoplantar pustulosis (PPP) is a chronic inflammatory disorder characterized by sterile pustules predominantly involving the palms and soles of middle-aged women. In contrast, regions other than the palms and soles are occasionally affected, manifesting scaly erythemas which resemble psoriasis, and solitary pustules are also seen. Some of these extra-palmoplantar lesions are induced by the Koebner phenomenon or occur after focal infections like tonsillitis. The tenderness and inflammation of the extra-palmoplantar lesions in PPP are milder than in psoriasis. Histological features show mild acanthosis of the epidermis with parakeratosis and mild infiltration of inflammatory cells in the upper dermis. On the other hand, severe pustular lesions are occasionally seen in the palms and soles of the patients with pustular psoriasis. These findings suggest a close relationship between PPP and psoriasis; however, different genetic, environmental, and immunological factors are likely to be involved. Recently, understanding of psoriasis pathophysiology has greatly progressed, and the concept of psoriasis pathogenesis is currently viewed as complicated responses between infiltrating leucocytes and the resident skin, via a number of inflammatory cytokines, chemokines, and mediators produced in the skin under regulation of cellular immune systems. By contrast, the pathogenesis of PPP has been poorly investigated. This paper reviews findings of the clinicopathophysiology of PPP, making a focus on the extra-palmoplantar lesions.
Palmoplantar pustulosis (PPP) discloses some differences compared to vulgar psoriasis (PV) in terms of age of onset, female predominance and low occurrence of psoriasis lesions elsewhere. Cigarette smoking has been associated to PPP in international studies; nevertheless, these studies were never performed among Brazilian.
To compare prevalence of smoking among PPP, PV and other dermatologic patients (NPD).
Case-control study involving 25 PPP patients from a reference psoriasis centre. Two control groups were matched according to gender and age: 50 patients with PV and 50 NPD. Confounders were adjusted by conditional multiple logistic regression.
Among cases, 84.0% were female and PPP age of disease onset (41.4 years) was greater than PV (34.5 years). Prevalence of ever smoking was higher among cases (92.0%) than PV (52.0%) and NPD (30.0%). Adjusted odds ratio of PPP ever smoking compared to PV and NPD was 9.5 and 36.2, respectively. All smokers reported the onset of their habit before the development of PPP.
There was significant association between PPP and smoking. However, the impact of giving it up in the clinical course of the disease remains to be established.
Palmo-plantar psoriasis (PPP) is a disabling condition that significantly impairs quality of life. PPP tends to be resistant to conventional therapies and may last for several years. Topical treatments are usually ineffective. Systemic therapy with oral retinoids and psoralen plus ultraviolet A is frequently required, although it rarely leads to remission.
We conducted an open-label, pilot study to evaluate treatment of PPP with efalizumab, an anti-CD11a monoclonal antibody approved for the treatment of chronic, refractory moderate to severe plaque psoriasis in adults.
Five patients with severe PPP received efalizumab treatment for 24 weeks.
All five patients responded favourably by week 12 and showed further improvement at week 24 of uninterrupted therapy. Mean physician-assessed severity scores and patient-reported outcome scores improved almost 75% after 12 weeks and 90% after 24 weeks. At week 32, three patients maintained the response seen at week 24, while two patients suspended efalizumab.
Efalizumab therapy was well tolerated and effective in five patients with severe PPP, allowing a significant improvement in quality of life.
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Abnormalities of the nail are not a well recognized feature of palmoplantar pustulosis (PPP). However, in a group of 50 patients with PPP, we found nail dystrophy in 15 (30%). The most frequent pattern was subungual pustulation, present in 10 patients, and progressing to nail destruction in two. Onycholysis and pitting of the nail were present in a few patients. In contrast to psoriasis vulgaris, the rate of linear nail growth in PPP was no greater than in matched normal controls. This is another clinical feature of PPP that separates it from psoriasis vulgaris.
Pustulosis palmoplantaris (PPP) is a common chronic skin disease, which is very resistant to treatment. It is not known why the lesions are located in the palms and soles. There are few studies of the disease and in particular studies of the histology. Fifty-nine patients with PPP answered a questionnaire concerning their medical history and 39 of them were clinically examined. Biopsy specimens were taken from involved skin in 22 of the 39 patients and studied immunohistologically for tryptase+ mast cells, EG2+ eosinophils, lipocalin+ neutrophils and CD3+ T lymphocytes. The sweat gland and sweat duct were visualized with AE1/AE3 antibody (cytokeratins 1-8, 10, 14/15, 16, 19). In addition to neutrophils in the pustule and lymphocytes in the upper dermis, there were also large numbers of mast cells and eosinophils in the subpustular area. Numerous eosinophils were present in the pustule. The epidermal part of the eccrine duct was not detectable in any of the specimens from patients with PPP but was present in all of the nine control persons (including two smokers). The results indicate that the acrosyringium is involved in the inflammation and also that mast cells and eosinophils participate in a hitherto unknown way. Of the 39 patients clinically examined, two had previously diagnosed thyroid disease and two had gluten hypersensitivity. Seventeen had one or several abnormal serum concentrations of thyroid-stimulating hormone, thyroxin, antibodies against thyroglobulin or thyroperoxidase and 10 had immunoglobulin (Ig) A antibodies to gliadin. The mean +/- SD for serum IgA and for eosinophil cationic protein was increased. From the questionnaire the most notable finding was that 56 of the 59 patients had been or still were smokers, all of whom had started smoking before the first signs of PPP. We hypothesize that the acrosyringium might be the target for the inflammation and that PPP is linked to autoimmune thyroid disease and smoking.
A suggested role for nicotine in the pathogenesis of palmoplantar pustulosis (PPP) has been discussed. The target for the inflammation in PPP is the acrosyringium. Nicotine acts as an agonist on nicotinic acetylcholine receptors (nAChRs) and can influence a variety of cellular functions.
To study the alpha 3- and alpha 7-nAChR expression in palmar skin of patients with PPP in comparison with that in healthy smoking and non-smoking controls.
Biopsies from 20 patients with PPP, seven healthy smokers and eight healthy non-smokers were studied by immunohistochemistry with a monoclonal anti-alpha 3 and a polyclonal anti-alpha 7 antibody.
In healthy controls both nAChR subtypes showed stronger immunoreactivity in the eccrine glands and ducts than in the epidermis. The papillary endothelium was positive for both subtypes. Epidermal alpha 3 staining was stronger and that of the coil and dermal ducts weaker in healthy smokers than in healthy non-smokers. In involved PPP skin, granulocytes displayed strong alpha 3 immunoreactivity. The normal epidermal alpha 7 staining pattern was abolished in PPP skin and was replaced by strong mesh-like surface staining, most markedly adjacent to the acrosyringium, which in controls was intensely alpha 7 positive at this level. Endothelial alpha 7 staining was stronger in PPP skin than in the controls.
Smoking can influence nAChR expression. The altered nAChR staining pattern in PPP skin may indicate a possible role for nicotine in the pathogenesis of PPP. We hypothesize that there is an abnormal response to nicotine in patients with PPP, resulting in inflammation.
The PSORS1 locus in the major histocompatibility complex region is the major genetic determinant for psoriasis vulgaris. Within the PSORS1 region reside at least three potential candidate genes for psoriasis susceptibility. Specific allelic variants of the genes HLA-Cw*6, HCR*WWCC, and CDSN*5 are strongly associated with psoriasis vulgaris and are in strong linkage disequilibrium with each other. We have genotyped the three psoriasis vulgaris susceptibility alleles of the PSORS1 locus in two clinical variants of psoriasis (guttate psoriasis and palmoplantar pustulosis) to study whether PSORS1 is also involved in the pathogenesis of these variants. We also asked whether these two clinical subgroups could help us to distinguish the causative gene within the high-risk PSORS1 haplotype. The association of guttate psoriasis with the three PSORS1 susceptibility alleles was similar and even stronger than seen with psoriasis vulgaris. Palmoplantar pustulosis, however, did not show association with any of the three candidate genes at this locus. Finally, no correlation with the age of onset for disease was observed. Our results show conclusively that psoriasis vulgaris and guttate psoriasis have a similar genetic basis for their association to PSORS1, whereas palmoplantar pustulosis appears to be a distinct disorder.
Palmoplantar pustulosis (PPP) is a skin disease characterized by chronically recurring sterile pustules on the palms and the soles. Although the aetiology of PPP is unknown, it is interesting to note the high prevalence of tobacco use in these patients. It would seem that there may be a relationship between PPP, autoimmune diseases and alterations of thyroid function.
We studied a total of 17 patients with ages ranging from 28 to 67 years, diagnosed with PPP. Patients were interviewed about: autoimmune diseases, psoriasis and thyroid disease, tobacco use (classified as: A, non-smoker; B, ex smoker; C, smoker of less than 20 cigarettes/day; D, smoker of more than 20 cigarettes/day). The patients were interviewed regarding the possible existence of a personal or family history of thyroid disease, determined using thyroid-stimulating hormone, thyroxine, antithyroid antibodies (antimicrosomal and antithyroglobulin antibodies).
The majority of the patients smoked cigarettes (according to classification). Of the 12 patients for whom a thyroid study was performed three had been diagnosed with thyroid diseases, an increase in thyroid-stimulating hormone in one case and two showed an increase in antimicrosomial antibodies.
We have been able to demonstrate a high prevalence of thyroid dysfunction and tobacco use in patients with PPP.
Psoriasis is a chronic, unpredictable, and incurable disease that has a negative impact on patients' quality of life. Palm and sole psoriasis can add to this negative impact as it directly affects activities of daily living.
We sought to estimate the prevalence of palmoplantar psoriasis in a patient population and to explore associations with patient outcomes.
In all, 317 individuals with psoriasis completed a comprehensive assessment battery. Patients with palmoplantar psoriasis (n = 124, 39%) were compared with patients without palmoplantar involvement with respect to functional disability, psychiatric symptoms, physical and social discomfort, self-reported psoriasis severity, and health-related quality of life.
Patients with palmoplantar involvement reported significantly greater physical disability and physical discomfort than patients without palmoplantar involvement (both P <.01). There were no differences between the 2 groups with respect to psychosocial outcomes.
Patients with palmoplantar psoriasis are affected to a greater degree by the physical aspects of the disease than patients without palmoplantar involvement.
Antigliadin antibodies (AGA) have been reported in patients with psoriasis.
To determine if AGA and other coeliac disease (CD)-associated antibodies correlate with clinical features and activity in patients with psoriasis.
Patients with psoriasis (n = 130) were investigated for serum IgG and IgA AGA, IgA antitransglutaminase antibody and IgA antiendomysial antibody. Disease characteristics and associated bowel and joint symptoms were determined. All patients were invited to undertake endoscopy with duodenal biopsy.
A significantly higher proportion of patients with elevated CD-associated antibody levels was currently on or had previously required systemic immunosuppressants (methotrexate, ciclosporin or etretinate; P = 0.04) or psoralen plus ultraviolet A phototherapy (P = 0.03). One case of CD was diagnosed.
The presence of CD-associated antibodies in psoriasis patients correlates with greater disease activity.
Background:
Chronic palmoplantar pustulosis (PPP) is a chronic inflammatory skin condition characterised by crops of sterile pustules (yellow pus spots) on the palms and soles which erupt repeatedly over months or years. The affected areas tend to become red and scaly; cracks may form and these are often painful. Many different treatments have been used for palmoplantar pustulosis but none is generally accepted as being reliably effective.
Objectives:
To assess the effects of treatments for palmoplantar pustulosis, both in reducing disease severity and in maintaining remission once achieved.
Search strategy:
We searched the Cochrane Skin Group Specialised Register (January 2003), the Cochrane Central Register of Controlled Trials (The Cochrane Library Issue 1, 2003), MEDLINE (1966 to February 2003), EMBASE (1988 to February 2003). We also cross-checked with the Salford Database of Psoriasis Trials and reference lists of articles. We also contacted authors included trials, members of the Cochrane Skin Group and dermatologists interested in psoriasis.
Selection criteria:
Any randomised controlled trial in which patients with chronic palmoplantar pustulosis were randomised to receive one or more interventions.
Data collection and analysis:
At least two reviewers independently assessed trial eligibility and quality. Study authors were contacted for additional information. Adverse effects information was collected from the trials.
Main results:
Twenty-three trials involving 724 people were included. There is evidence supporting the use of systemic retinoids (improvement rate difference 44%, 95 CI 28 to 59%), oral PUVA (improvement rate difference 44%, 95 CI 26 to 62%). However, a combination of PUVA and retinoids is better than the individual treatments. The use of topical steroid under hydrocolloid occlusion is beneficial. It would also appear that low dose ciclosporin, tetracycline antibiotics and Grenz Ray Therapy may be useful in treating PPP. Colchicine has a lot of side effects and it is unclear if it is effective and neither was topical PUVA (rate difference of 0.00, 95% CI -0.04 to +0.04). There is no evidence to suggest that short-term treatment with hydroxycarbamide (hydroxyurea) is effective.
Authors' conclusions:
Many different interventions were reported to produce "improvement" in PPP. There is, however, no standardised method for assessing response to treatment, and reductions in pustule counts or other empirical semi-quantitative scoring systems may be of little relevance to the patient. This review has shown that the ideal treatment for PPP remains elusive and that the standards of study design and reporting need to be improved to inform patients and those treating them of the relative merits of the many treatments available to them.
A 50-year-old woman developed prominent hyperkeratosis on the soles and erythema of her extremities. In this instance it was clinically difficult to distinguish the palmoplantar lesions from psoriasis vulgaris. However, our case proved to be palmoplantar pustulosis (PPP) because, on histology, the extra-palmoplantar lesions showed none of the typical findings of psoriasis vulgaris, there was a history of sternoclavicular joint pain, and the lesions improved after tonsillectomy. Herein, we consider the relationship between prominent hyperkeratosis of the sole or palm in PPP and bacterial infection.