Article

Clinical and epidemiological comparison of patients affected by palmoplantar plaque psoriasis and palmoplantar pustulosis: A case series study

January 2013
British Journal of Dermatology 168(6)

DOI:10.1111/bjd.12223

Source
PubMed

Authors:

Matteo Puntoni

Galliera Hospital

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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.

Clinical Characteristics, Quality of Life and Risk Factors for Severity in Palmoplantar Pustulosis: A Cross‐Sectional, Multicentre Study of 263 Patients

Article

Full-text available

May 2022

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.

Prevalence and incidence of generalised pustular psoriasis in Sweden – a population‐based register study

Article

Full-text available

Jan 2022

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.

Pustular Psoriasis: From Pathophysiology to Treatment

Article

Full-text available

Nov 2021

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.

Prevalence and incidence of palmoplantar pustulosis in Sweden: a population‐based register study

Article

Full-text available

Apr 2021

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.

Clinical characteristics, genetics, comorbidities and treatment of palmoplantar pustulosis: A different perspective

Article

Nov 2020

Palmoplantar Pustulosis: A Case Report

Article

Full-text available

Sep 2020

Introduction: Dermatology complaints account for 3.3% of emergency department (ED) visits per year. Most rashes are benign, but there are a select few that emergency physicians must be familiar with as delay in treatment could be life threatening. Case report: A well-appearing, 76-year-old male presented to the ED with multiple coalescing pustules to his palms and soles and was transferred to the nearest tertiary care hospital for dermatology consult. He was diagnosed with palmoplantar pustulosis and discharged home with a five-day course of clobetasol propionate 0.05% cream twice daily and outpatient dermatology follow-up. Conclusion: Palmoplantar pustulosis is an uncommon skin condition characterized by recurrent eruptions of sterile pustules localized to the palms and soles. Emergency physician awareness of this rare diagnosis may help prevent hospital admissions and lead to earlier initiation of treatment with outpatient dermatology follow-up.

Diagnosis, Screening and Treatment of Patients with Palmoplantar Pustulosis (PPP): A Review of Current Practices and Recommendations

Article

Full-text available

Aug 2020

Palmoplantar pustulosis (PPP) is a rare, chronic, recurrent inflammatory disease that affects the palms and/or the soles with sterile, erupting pustules, which are debilitating and usually resistant to treatment. It has genetic, histopathologic and clinical features that are not present in psoriasis; thus, it can be classified as a variant of psoriasis or as a separate entity. Smoking and upper respiratory infections have been suggested as main triggers of PPP. PPP is a challenging disease to manage, and the treatment approach involves both topical and systemic therapies, as well as phototherapy and targeted molecules. No gold standard therapy has yet been identified, and none of the treatments are curative. In patients with mild disease, control may be achieved with on-demand occlusion of topical agents. In patients with moderate-to-severe PPP, phototherapy or a classical systemic agent (acitretin being the best treatment option, especially in combination with PUVA) may be effective. Refractory patients or those with contraindications to use these therapies may be good candidates for apremilast or biologic therapy, particularly anti-IL-17A and anti-IL-23 agents. Recent PPP trials are focusing on blockage of IL-36 or IL-1 pathways, which play an important role in innate immunity. Indeed, IL-36 isoforms have been strongly implicated in the pathogenesis of psoriasis. Therefore, blockage of the IL-36 pathway has become a new treatment target in PPP, and three studies are currently evaluating the use of monoclonal antibodies that block the IL-36 receptor in PPP: ANB019 and spesolimab (BI 655130). In this review, we explore the diagnosis, screening and treatment of patients with PPP.

The clinical, humanistic, and economic burden of palmoplantar pustulosis: a structured review

Article

Full-text available

Feb 2020

Introduction: Palmoplantar pustulosis (PPP) is a chronic, relapsing and refractory disease characterized by sterile pustules appearing on the palms and/or soles, accompanied by erythema, blistering, scales and/or keratinization. The overall burden of PPP in terms of its clinical impact, effect on patients and families, and economic consequences has not previously been investigated in a structured manner. Areas covered: A structured search focused on identification of studies in PPP using specific search terms in PubMed and EMBASE® from 2005 onwards, with additional back-referencing and pragmatic searches. Outcomes of interest included clinical burden, humanistic burden, and economic burden. Expert opinion: In cross-sectional studies, approximately 75% of all PPP patients suffer from active disease, with risk of relapse remaining constant over time. Patients’ health-related quality of life is significantly impaired, as expected for a disease affecting hands and feet. Tools have been described that assess the clinical as well as patient-reported burden of PPP; their performance in larger cohorts and/or clinical trials remains to be investigated. The key data limitations identified include inconsistent definitions for characterizing remission/relapse, and limited humanistic and economic burden data; future studies are required to address these evidence gaps.

Palmoplantar Pustulosis: Recent Advances in Etiopathogenesis and Emerging Treatments

Article

Full-text available

Feb 2020

Palmoplantar pustulosis (PPP) is a chronic, recurrent skin disease belonging to the spectrum of psoriasis. It is characterized by an eruption of sterile pustules on the palms and soles. Recent studies in PPP have focused on genetic differences between pustular phenotypes and the role of the innate immunological system and the microbiome in the etiopathogenesis of the disease. Mutations in IL36RN (a major predisposing factor for generalized pustular psoriasis) were found in selected patients with PPP and were associated with earlier disease onset. Studies have shown that the interleukin (IL)-17 and IL-36 pathways might be involved in the pathogenesis of PPP. A microbiome has been demonstrated in the vesicopustules of PPP, and an abundance of Staphylococcus appears to be increased by smoking. Improved understanding of the underlying etiopathogenesis of PPP has led to advances in treatment options, and targeted therapies for PPP have been evaluated or are under evaluation against more than 12 molecules in ongoing clinical trials. These targets include CXCR2 (IL-8 receptor type B), granulocyte colony-stimulating factor receptor, IL-1 receptor, IL-8, IL-12, IL-23, IL-17A, IL-17 receptor, IL-36 receptor, phosphodiesterase-4, and tumor necrosis factor-α.

Palmoplantar Plaque Psoriasis is Associated with Diabetes, Hypertension, Obesity, and Metabolic Syndrome-A Case-Control Study

Article

Full-text available

Sep 2022

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.

CNVs Associated with Different Clinical Phenotypes of Psoriasis and Anti-TNF-Induced Palmoplantar Pustulosis

Article

Full-text available

Sep 2022

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.

Efficacy of narrowband phototherapy in the treatment of different forms of psoriasis with the predominant affection of palms and soles

Article

Full-text available

Dec 2021

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.

MrgprC11 + sensory neurons mediate glabrous skin itch

Article

Apr 2021

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.

Palmoplantar pustulosis: Current understanding of disease definition and pathomechanism

Article

Full-text available

Mar 2020
J DERMATOL SCI

Pustulosis palmaris et plantaris, or palmoplantar pustulosis (PPP), is a chronic pustular dermatitis involving the palms and soles and is characterized by vesicles, pustules, erythema, lichenification, and abnormal desquamation. It is one of the most common skin diseases in Japan but its pathomechanism is unclear and the disease remains poorly defined. Consequently, adequate treatment for PPP is lacking. As a localized type of pustular psoriasis, PPP has long been treated with the conventional therapies used for plaque-type psoriasis, especially in Western countries. However, PPP may be a distinct entity, with a much lower prevalence in Western countries than in Japan. Furthermore, while treatment has yielded insights into the underlying pathology in plaque-type psoriasis, the pathogenesis of PPP has yet to be elucidated. In 2018, Gulselkumab, a monoclonal antibody against interleukin (IL)-23, was certified for use in Japan and is the first biologic effective in PPP both in Japanese and other patients. In this review, we summarize the current understanding of PPP, including the revised definition and possible pathomechanism. The information presented herein provides a more complete picture of PPP and may facilitate the development of improved treatment options.

Characterization of Patients with Psoriasis in Challenging-to-Treat Body Areas in the Corrona Psoriasis Registry

Article

Full-text available

Jan 2020

Background: Real-world studies evaluating patients with challenging-to-treat localizations of psoriasis (scalp, nail, and palmoplantar) are limited. Objective: To characterize patients with versus without psoriasis in challenging-to-treat areas seen in routine US clinical practice. Methods: This retrospective observational study included all adult patients with psoriasis enrolled in the Corrona Psoriasis Registry between April 2015 and May 2018 who initiated a biologic therapy at registry enrollment. Patients were stratified by the presence of scalp, nail, or palmoplantar psoriasis (nonmutually exclusive groups). Patient demographics, clinical char-acteristics, disease activity, and patient-reported outcome measures (pain, fatigue, itch, EuroQol visual analog scale [EQ VAS], Dermatology Life Quality Index [DLQI], and Work Productivity and Activity Impairment questionnaire [WPAI]) were assessed at registry enrollment and compared between patients with versus without each challenging-to-treat area using nonparametric Kruskal-Wallis tests for continuous variables and χ2 or Fisher exact tests for categorical variables. Generalized linear regression models were used to estimate differences in disease activity and patient-reported outcomes between patients with versus without each challenging-to-treat area. Results: Among 2,042 patients with psoriasis (mean age [±SD], 49.6 ± 14.7 years; 51.5% male), 38.4% had psoriatic arthritis (PsA), 38.1% had scalp psoriasis, 16.0% had nail psoriasis, 10.9% had palmoplantar psoriasis, and 26.2% had a combination of ≥2 challenging-to-treat areas and PsA; only 34.2% had body plaque psoriasis without PsA or challenging-to-treat areas. Patients in all challenging-to-treat groups reported higher (mean [95% CI]) itch (scalp, 58.01 [57.62-58.40] vs. 54.35 [53.99-54.72]; nail, 56.42 [56.02-56.81] vs. 55.59 [55.20-55.97]; palmoplantar, 60.22 [59.86-60.59] vs. 55.15 [54.79-55.54]) and lower EQ VAS (scalp, 68.12 [67.78-68.48] vs. 69.46 [69.12-69.81]; nail, 66.21 [65.89-66.55] vs. 69.48 [69.14-69.83]; palmoplantar, 66.21 [66.07-66.75] vs. 69.29 [68.94-69.94]) scores than those without the respective challenging-to-treat localization. Patients with nail or palmoplantar psoriasis reported higher pain, fatigue, and DLQI scores than those without. Higher proportions of patients with scalp or palmoplantar psoriasis reported work impairment compared with those without. Conclusion: Two-thirds of patients with psoriasis who initiated biologic therapy had PsA and/or ≥1 challenging-to-treat area. Patients with challenging-to-treat areas had worse patient-reported outcome scores than those without, indicating a significant burden of challenging-to-treat areas on patients' quality of life.

Pustular psoriasis: A distinct aetiopathogenic and clinical entity

Article

Full-text available

May 2023
INDIAN J DERMATOL VE

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.

Economic Burden of Palmoplantar Pustulosis in Sweden: A Population-based Register Study

Article

Full-text available

Jan 2023
ACTA DERM-VENEREOL

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.

Atualizações sobre a patogênese, as manifestações clínicas e o diagnóstico da Psoríase Pustular: Updates on the pathogenesis, clinical manifestations and diagnosis of Pustular Psoriasis

Article

Dec 2022

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.

Narrative Review of the Emerging Therapeutic Role of Brodalumab in Difficult-to-Treat Psoriasis

Article

Full-text available

Jun 2022

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.

Psoriasis in Saudi Population: Gender Differences in Clinical Characteristics and Quality of Life

Article

Full-text available

Mar 2022

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.

Efficacy of transdermal methotrexate iontophoresis and low dose oral methotrexate in the treatment of palmoplantar psoriasis

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Clinical characteristics and treatment status of pustulotic arthro‐osteitis: A single‐center study involving 51 cases

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Full-text available

Aug 2021

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.

Pustular Psoriasis and Associated Musculoskeletal Disorders

Article

Mar 2021

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.

Assessment of efficacy of methotrexate and acitretin in the management of psoriasis

Article

Jul 2019

Successful Treatment of Refractory Palmoplantar Pustular Psoriasis With Apremilast: A Case Series

Article

Full-text available

Oct 2020

Introduction: Palmoplantar pustular psoriasis (PPPP) is a debilitating inflammatory skin disorder of the palms and soles that poses a high burden on affected patients. Satisfactory treatment response is rarely achieved using current treatment options, little is known about the potential benefit of the PDE4 inhibitor apremilast in the treatment of refractory PPPP patients. We aimed to evaluate the use of apremilast in PPPP patients. Patients and Methods: Six patients, four with severe physician global assessment (PGA) = 3 on a scale of 0–4 and two with very severe (PGA = 4) treatment-refractory PPPP [mean age (years ± SD): 56.2 ± 15.6], were included in this study. Five patients had concomitant psoriatic arthritis (PsA). Prior to apremilast administration, topical corticosteroids, psoralen-UVA and multiple systemic oral and biologic anti-inflammatory treatments were insufficient to improve their skin condition or had to be discontinued due to adverse events. Apremilast (titrated to a maintenance dose of 30 mg 2x/d) was commenced in all patients with clinical follow-up over 18 months. Results: Within the first 4 weeks of treatment, each patient's symptoms improved as assessed by PGA score. At 3 months, four patients had a mild PGA score and two were cleared from PPPP. After 18 months of follow-up, three patients improved from PGA = 3 to PGA = 1 and one patient from PGA = 4 to PGA = 1. Two patients discontinued treatment, one due to a lack of efficacy against PsA and the other to a desire to have a child. However, both patients recorded improvements before discontinuing treatment. Conclusion: Apremilast may be a promising treatment option for refractory and severely affected PPPP patients. Our observation, however, requires further validation.

Association of Clinical and Demographic Factors With the Severity of Palmoplantar Pustulosis

Article

Full-text available

Sep 2020

Importance: Although palmoplantar pustulosis (PPP) can significantly impact quality of life, the factors underlying disease severity have not been studied. Objective: To examine the factors associated with PPP severity. Design, setting, and participants: An observational, cross-sectional study of 2 cohorts was conducted. A UK data set including 203 patients was obtained through the Anakinra in Pustular Psoriasis, Response in a Controlled Trial (2016-2019) and its sister research study Pustular Psoriasis, Elucidating Underlying Mechanisms (2016-2020). A Northern European cohort including 193 patients was independently ascertained by the European Rare and Severe Psoriasis Expert Network (2014-2017). Patients had been recruited in secondary or tertiary dermatology referral centers. All patients were of European descent. The PPP diagnosis was established by dermatologists, based on clinical examination and/or published consensus criteria. The present study was conducted from October 1, 2014, to March 15, 2020. Main outcomes and measures: Demographic characteristics, comorbidities, smoking status, Palmoplantar Pustulosis Psoriasis Area Severity Index (PPPASI), measuring severity from 0 (no sign of disease) to 72 (very severe disease), or Physician Global Assessment (PGA), measuring severity as 0 (clear), 1 (almost clear), 2 (mild), 3 (moderate), and 4 (severe). Results: Among the 203 UK patients (43 men [21%], 160 women [79%]; median age at onset, 48 [interquartile range (IQR), 38-59] years), the PPPASI was inversely correlated with age of onset (r = -0.18, P = .01). Similarly, in the 159 Northern European patients who were eligible for inclusion in this analysis (25 men [16%], 134 women [84%]; median age at onset, 45 [IQR, 34-53.3] years), the median age at onset was lower in individuals with a moderate to severe PGA score (41 years [IQR, 30.5-52 years]) compared with those with a clear to mild PGA score (46.5 years [IQR, 35-55 years]) (P = .04). In the UK sample, the median PPPASI score was higher in women (9.6 [IQR, 3.0-16.2]) vs men (4.0 [IQR, 1.0-11.7]) (P = .01). Likewise, moderate to severe PPP was more prevalent among Northern European women (57 of 134 [43%]) compared with men (5 of 25 [20%]) (P = .03). In the UK cohort, the median PPPASI score was increased in current smokers (10.7 [IQR, 4.2-17.5]) compared with former smokers (7 [IQR, 2.0-14.4]) and nonsmokers (2.2 [IQR, 1-6]) (P = .003). Comparable differences were observed in the Northern European data set, as the prevalence of moderate to severe PPP was higher in former and current smokers (51 of 130 [39%]) compared with nonsmokers (6 of 24 [25%]) (P = .14). Conclusions and relevance: The findings of this study suggest that PPP severity is associated with early-onset disease, female sex, and smoking status. Thus, smoking cessation intervention might be beneficial.

Treatment patterns and healthcare resource utilization in palmoplantar pustulosis patients in Japan: A claims database study

Article

Full-text available

May 2020
PLOS ONE

Background Palmoplantar pustulosis (PPP) is a chronic, relapsing, inflammatory autoimmune condition, characterized by sterile pustules on the palms and soles. The treatment patterns of PPP and total health care resource utilization in Japan are not well described. Investigating these areas is needed to understand current PPP management in Japan. Objective To describe the characteristics, medication treatment and health care resource utilization patterns, and associated costs of PPP patients in Japan. Methods A retrospective analysis of insurance claims data was conducted using the Japan Medical Data Center database. Adult Patients with at least two claims with a PPP diagnosis from January 1, 2011 to March 30, 2017 and six months of follow-up after the first diagnosis were included. Patient characteristics described include age, gender, and comorbid conditions. Treatment patterns assessed include the types of treatment, sequence of treatment, and rates of discontinuation, switching, persistence and use of concomitant medications. Results A total of 5,162 adult patients met all inclusion criteria. Mean (SD) patient age was 49.7 (11.6) years and 43.2% were male. A total of 2441 patients (47.8%) received systemic non-biologic drugs during the entire follow up period, 2,366 (46.4%) were prescribed topical therapy, 273 (5.4%) were prescribed phototherapy, while 18 (0.4%) of patients with other autoimmune comorbidities were eligible for prescribed biologics. For treatment-naïve patients with mild PPP, topical therapy was most commonly (77.1%) prescribed, whereas in moderate to severe cases of PPP, systemic non-biologic drugs (65%) were most often used. The frequency of switching was similar (64.3% to 75.3%) across various therapies and treatment lines. Conclusion This study describes the treatment patterns and health care resource utilization for Japanese PPP patients using a large claims database, and highlights an unmet need to derive better treatment strategies for PPP and address disease burden in Japan.

Characteristics and Burdens of Disease in Patients from Beijing with Generalized Pustular Psoriasis and Palmoplantar Pustulosis: Multicenter Retrospective Cohort Study Using a Regional Database

Article

Jun 2023
AM J CLIN DERMATOL

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.

An update on therapeutic options for palmoplantar pustulosis: a narrative review and expert recommendations

Article

Mar 2023
Expet Rev Clin Immunol

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.

Evaluation of the area subscore of the Palmoplantar Pustulosis Area and Severity Index using an attention U-net deep learning algorithm

Article

Feb 2023
J DERMATOL

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.

Comparison of the Inflammatory Circuits in Psoriasis Vulgaris, Non-Pustular Palmoplantar Psoriasis, and Palmoplantar Pustular Psoriasis

Article

Aug 2022
J INVEST DERMATOL

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.

Differences in epidemiology, comorbidities and treatment choice between plaque psoriasis and pustular psoriasis: results from the BIOBADADERM registry

Article

Full-text available

Jul 2022

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.

Pustulose Palmo-Plantar

Article

Full-text available

Jun 2022

Risks of Comorbidities in Patients With Palmoplantar Pustulosis vs Patients With Psoriasis Vulgaris or Pompholyx in Korea

Article

Apr 2022

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.

Biologic therapies associated with development of palmoplantar pustulosis and palmoplantar pustular psoriasis: a systematic review

Article

Feb 2022
INT J DERMATOL

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.

Evaluation of a Case Series of Patients With Palmoplantar Pustulosis in the United States

Article

Dec 2021

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.

Pharmacokinetic/Pharmacodynamic Analysis of Guselkumab for Treatment of Palmoplantar Pustulosis: Clinical Implications of Guselkumab Dose, Disease Severity and Smoking in Japanese Patients

Article

Aug 2021

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

Recent advances in palmoplantar pustulosis

Article

Full-text available

Jul 2021

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.

SnapshotDx Quiz: May 2021

Article

May 2021
J INVEST DERMATOL

Comparison of the Efficacy and Safety of Apremilast and Methotrexate in Patients with Palmoplantar Psoriasis: A Randomized Controlled Trial

Article

Mar 2021

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.

Rapid therapeutic response of palmoplantar pustulosis under biologic treatment with guselkumab

Article

Full-text available

Feb 2021
DERMATOL THER

Psoriasis - das kann der Hausarzt tunPsoriasis

Article

Nov 2020

Palmoplantar psoriasis

Chapter

Apr 2017

Interventions for chronic palmoplantar pustulosis: abridged Cochrane systematic review and GRADE assessments

Article

Sep 2020
BRIT J DERMATOL

Background Palmoplantar pustulosis (PPP) is a chronic inflammatory disease in which sterile and relapsing pustules appear on the palms and soles. Objectives To assess the effects of interventions for chronic PPP to induce and maintain complete remission. Methods We searched for randomised controlled trials (RCTs), including people with PPP or chronic palmoplantar pustular psoriasis, in Cochrane Skin Specialised Register, CENTRAL, MEDLINE, Embase, LILACS and eight trials registers up to July 2020. Study selection, data extraction and risk of bias assessment were carried out independently by two review authors. Certainity of evidence was assessed using the GRADE (Grading of Recommendations Assessment, Development and Evaluation) method. Results We included 37 RCTs (1663 participants, 76% women, mean age 50 years).. Mean treatment duration was 11 weeks. Topical vitamin D derivative may be more effective than placebo in achieving clearance [risk ratio (RR) 7.83, 95% confidence interval (CI) 1.85 to 33.12; low‐certainity evidence from 2 trials].. Concerning biologic therapies, there was little or no difference between etanercept and placebo in achieving clearance (low‐certainity evidence from one trial), ustekinumab is less effective than placebo in reducing severity (low‐certainty evidence from one trial), guselkumab and secukinumab probably better reduce disease severity (RR 2.88, 95% CI 1.24 to 6.69 and 1.55, 95% CI 1.02 to 2.35 respectively, moderate‐certainty evidence from 2 and one trial respectively) but may cause more serious adverse events when compared to placebo. Conclusions Evidence is lacking for or against major chronic PPP treatments. Risk of bias and imprecision limit our confidence in the results.

Treatment of Palmoplantar Pustulosis Using Fixed Combination Halobetasol Propionate 0.01% and Tazarotene 0.045% Lotion: A Case Report

Article

Aug 2020

Background Psoriasis is a chronic inflammatory disease that affects skin and joints and is associated with numerous comorbidities. There are several clinical subtypes including the uncommon pustular variants, which are subdivided into generalized and localized forms. Palmoplantar pustulosis is considered a subtype of pustular psoriasis that manifests as localized eruptions of pustules affecting the palms and/or soles. The disease often persists for several years or decades, with frequent periods of exacerbation and partial remissions and may significantly impair quality of life secondary to pain, pruritus and resulting functional disability. Case Description This case report documents a 54-year-old woman who presented with a painful pustular lesion on her right palm. Careful patient history and biopsy of the lesion confirmed palmoplantar pustulosis. Initial treatment with clobetasol propionate 0.25% cream resulted in no improvement so she was switched to halobetasol propionate 0.01% and tazarotene 0.045% lotion—a novel corticosteroid–retinoid combination, while awaiting payor approval for systemic biologic therapy. Within a week, the pustular lesions and pain had resolved and her quality of life was significantly improved, so the patient elected to continue with topical therapy. This improvement was maintained at 6-week follow-up and during a 2-week reduced dose treatment schedule. She reflared 5 weeks after stopping treatment, but cleared again after reintroduction of the therapy. No side effects or adverse events were reported during treatment. Conclusion Halobetasol propionate 0.01% and tazarotene 0.045% lotion may be an effective option for treatment and maintenance in patients with palmoplantar pustulosis.

Comorbidities in patients with palmoplantar plaque psoriasis

Article

Aug 2020
J AM ACAD DERMATOL

Background Psoriasis has been shown to be associated with several comorbidities. Whether the palmoplantar subtype of plaque psoriasis carries similar risks for comorbidities as generalized plaque psoriasis remains to be defined. Objective To examine the association between palmoplantar plaque psoriasis and comorbidities known to be associated with generalized plaque psoriasis. Methods We retrospectively compared the prevalence of comorbidities previously found to be associated with generalized plaque psoriasis among 163 patients with palmoplantar plaque psoriasis who had been treated with topical psoralen and ultraviolet A from 2009 to 2017 and a cohort of 781 control individuals. Each patient with psoriasis was matched according to sex and age (±1 year) with up to 5 control individuals. Conditional logistic regression was used to evaluate the associations after matching. Results Diabetes mellitus (odds ratio [OR], 2.296), cardiovascular disease (OR, 1.797), and most remarkably, mood disorders (OR, 6.232) were significantly associated with palmoplantar plaque psoriasis. Dyslipidemia, hypertension, and psoriatic arthritis were more frequent among patients with palmoplantar plaque psoriasis, but those associations did not reach statistical significance. Limitations The retrospective nature of this study, the fact that some data were collected through a survey questionnaire, and the relatively small sample size suggest the need to validate the present data in a prospective manner. Additionally, within the psoriasis group, patients were assessed for the presence of comorbidities during the whole follow-up period, whereas the comorbidities of individuals in the control group were assessed during a baseline visit. Conclusions Several comorbidities known to be associated with psoriasis vulgaris were also found to be prevalent in a series of patients with plaque palmoplantar psoriasis. Individuals affected with plaque palmoplantar psoriasis showed a particularly high risk for mood disorders.

Efficacy of guselkumab in a subpopulation with pustulotic arthro‐osteitis through week 52: An exploratory analysis of a phase 3, randomized, double‐blind, placebo‐controlled study in Japanese patients with palmoplantar pustulosis

Article

Full-text available

Mar 2020

Background: Previous studies of guselkumab have demonstrated clinical benefits in patients with plaque-type psoriasis, generalized pustular psoriasis, erythrodermic psoriasis, and palmoplantar pustulosis (PPP). Objective: The aim of this exploratory analysis of a double-blind, multicenter, placebo-controlled, phase 3 study in Japanese patients with PPP was to evaluate the efficacy of guselkumab in the subset of patients with pustulotic arthro-osteitis (PAO). Methods: Patients were randomized to receive guselkumab 100 mg or 200 mg at weeks 0, 4, 12, and every 8 weeks, or placebo with cross-over to guselkumab 100 mg or 200 mg at week 16 (placebo group). Efficacy endpoints were changes from baseline in magnetic resonance imaging (MRI) score, EuroQOL-5 dimensions (EQ-5D) index score, EQ-5D pain/discomfort dimension score, and C-reactive protein (CRP, mg/L) level in all PAO patients through week 52. Data from both guselkumab groups were combined and presented as results for a single overall guselkumab group. Results: Among 159 patients with PPP, 66 with PAO were randomized across treatment groups. For patients with MRI data for all regions assessed, the proportion of patients in the guselkumab group with PAO characterized as severe decreased from 23.8% (10/42) at baseline to 5.4% (2/42) at week 52. The mean (SD) change from baseline at week 52 in EQ-5D index score was 0.20 (0.17) among PPP patients with PAO and 0.15 (0.17) among those without PAO in the guselkumab group. Among all PAO patients, the proportions with an EQ-5D pain/discomfort dimension score of no or slight pain/discomfort in the guselkumab group increased from baseline to week 52 (33.3% [7/21] vs 87.5% [35/40]). The mean (SD) CRP levels decreased in all PAO patients in the guselkumab group at week 52 compared to baseline (-1.71 [8.16] mg/L). Conclusion: Guselkumab treatment showed beneficial outcomes for PAO signs and symptoms in Japanese patients with PPP.

Sex‐ and gender‐associated clinical and psychosocial characteristics of patients with psoriasis

Article

Mar 2020

Background: Sex and gender may affect disease prevalence, adverse effects and response to therapy. Aim: To analyse sex- and gender differences in psoriasis outpatients. Methods: A cross-sectional study was conducted at IDI-IRCCS, Rome, over a 3-year period. 3023 psoriasis patients were enrolled. Anthropometric and demographic characteristics were recorded, and a dermatologist evaluated the clinical severity of disease. Quality of life questionnaires were collected. Univariate and multivariate analyses were performed to examine factors associated with sex. Results: We found sex- and gender- differences in clinical characteristics, severity of disease, psychological distress, and quality of life. Male sex was associated with Body Mass Index, smoking, alcohol consumption, Psoriasis Area Severity Index (PASI) ≥10, age at onset ≥20 years. Female sex was associated with family history of diabetes, joint involvement, clinical type other than plaque diffuse, higher psychological distress, and a greater burden on quality of life. Conclusion: Our study identified sex- and gender differences of potential clinical relevance.

Interventions for chronic palmoplantar pustulosis

Article

Jan 2020

Background: Palmoplantar pustulosis is a chronic inflammatory disease in which sterile, relapsing pustules appear on the palms and soles, possibly in conjunction with other symptoms. The previous Cochrane Review on this topic was published in 2006, before biological treatments were extensively used. Objectives: To assess the effects of interventions for chronic palmoplantar pustulosis to induce and maintain complete remission. Search methods: We searched the following databases up to March 2019: Cochrane Skin Specialised Register, CENTRAL, MEDLINE, Embase, and LILACS. We also searched five trials registers and checked the reference lists of the included studies for further references to relevant randomised controlled trials (RCTs). Selection criteria: We considered RCTs including people with palmoplantar pustulosis or chronic palmoplantar pustular psoriasis assessing topical therapy, systemic therapy, combinations of topical or systemic therapies, or non-pharmacological therapies compared with placebo, no intervention, or each other. Data collection and analysis: We used standard methodological procedures expected by Cochrane. Our outcomes included 'Proportion of participants cleared or almost cleared', 'Proportion of participants with adverse effects serious or severe enough to cause withdrawal', 'Proportion of participants with at least 50% improvement in disease severity', and 'Proportion of participants with adverse effects'. Main results: We included 37 studies (1663 participants; mean age 50 years (range 34 to 63); 24% males). These studies reported condition severity differently. Around half of the included trials stated the setting (hospitals, community clinics, or both). More than half of the studies were at high risk of bias in at least one domain. Our included studies assessed mainly systemic treatments (retinoids, ciclosporin, biologics, etretinate + PUVA (combination of psoralens and long-wave ultraviolet radiation) therapy combined, and antibiotics), but also topical treatments (dermocorticoids, vitamin D) and phototherapy (PUVA, ultraviolet A1 (UVA1)). Other interventions were assessed by single studies. The most common comparator was placebo. All results presented in this abstract were assessed in the short term (mean treatment duration was 11 weeks (range 8 to 24 weeks)) and are based on participants with chronic palmoplantar pustulosis. All outcome time point measurements were taken from baseline and assessed at the end of treatment. Short-term and long-term outcomes were defined as measurement up to 24 weeks after randomisation and between 24 and 104 weeks after randomisation, respectively. One trial (188 participants) assessed the topical vitamin D derivative maxacalcitol versus placebo and found that maxacalcitol may be more effective than placebo in achieving clearance (risk ratio (RR) 7.83, 95% confidence interval (CI) 1.85 to 33.12; low-quality evidence), and the risk of adverse effects (such as mild local irritation, pruritus, and haematological or urinary test abnormalities) is probably similar in both groups (RR 0.87, 95% CI 0.64 to 1.19; moderate-quality evidence). Severity was not reported. Two trials (49 participants) assessed PUVA therapy versus placebo or no treatment, providing very low-quality evidence. Adverse effects were reported with oral PUVA (including nausea, ankle swelling, and non-purulent conjunctivitis) and with local PUVA (including blistering, erythema, and pruritus). With regard to the systemic retinoid alitretinoin, one trial (33 participants; moderate-quality evidence) showed that alitretinoin probably makes little or no difference in reducing severity when compared to placebo (RR 0.69, 95% CI 0.36 to 1.30). A similar number of adverse events were reported in both treatment groups, including headache, cheilitis, nausea, arthralgia, and nasopharyngitis (RR 0.84, 95% CI 0.61 to 1.17). Clearance was not reported. There may be little or no difference between etanercept and placebo in achieving clearance (RR 1.64, 95% CI 0.08 to 34.28; 1 study; 15 participants; low-quality evidence); however, the 95% CI was very wide, showing there may be a difference between groups. Severity was not measured. More patients treated with placebo may achieve reduced severity than those treated with ustekinumab, but the wide 95% CI indicates there might be little or no difference between groups and there might be greater effect with ustekinumab (RR 0.48, 95% CI 0.11 to 2.13; 1 study; 33 participants; low-quality evidence). Clearance was not reported. It is uncertain whether guselkumab increases clearance when compared to placebo (2 studies; 154 participants) because the quality of evidence is very low, but guselkumab probably better reduces disease severity (RR 2.88, 95% CI 1.24 to 6.69; 1 study; 49 participants; moderate-quality evidence). Secukinumab is probably superior to placebo in reducing severity (RR 1.55, 95% CI 1.02 to 2.35; 1 study; 157 participants; moderate-quality evidence), but our clearance outcome was not reported. None of these trials reported on occurrence of adverse effects. Only two of the studies discussed above reported adverse effects serious or severe enough to cause withdrawal. Guselkumab may cause more serious adverse events when compared to placebo, but there is uncertainty due to the very wide 95% CI showing there may be little or no difference and showing more events with placebo (RR 2.88, 95% CI 0.32 to 25.80; 1 study; 49 participants; low-quality evidence). Secukinumab probably causes more serious adverse events than placebo (RR 3.29, 95% CI 1.40 to 7.75; 1 study; 157 participants; moderate-quality evidence). Authors' conclusions: Evidence is lacking for major chronic palmoplantar pustulosis treatments such as superpotent corticosteroids, phototherapy, acitretin, methotrexate, and ciclosporin. Risk of bias and imprecision limit our confidence. Maxacalcitol may be more effective than placebo in achieving clearance in the short term (low-quality evidence), and the risk of adverse effects is probably similar (moderate-quality evidence). Oral alitretinoin is probably no more effective than placebo in reducing severity, with a similar risk of adverse effects (moderate-quality evidence). Regarding biological treatments, we are uncertain of the effect of etanercept on clearance and the effect of ustekinumab on severity (low-quality evidence). Secukinumab and guselkumab are probably superior to placebo in reducing severity (moderate-quality evidence). Adverse events not requiring withdrawal were not reported for these treatments. Reporting of serious adverse effects was incomplete: compared to placebo, secukinumab probably caused more participant withdrawals (moderate-quality evidence), but we are uncertain of the effect of guselkumab (low-quality evidence). Future trials should assess commonly used treatments using validated severity and quality of life scales.

Efficacy of Ustekinumab in Palmoplantar Psoriasis

Article

Full-text available

Oct 2010
DERMATOLOGY

No abstract available.

Successful treatment of hand and foot psoriasis with infliximab

Article

Full-text available

Jul 2010
Dermatol Online J

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.

Pustulosis palmaris et plantaris. A follow-up study of a ten-year material

Article

Full-text available

Feb 1971

Palmoplantar Lesions in Psoriasis: A Study of 3065 Patients

Article

Full-text available

Feb 2002

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.

Palmoplantar Pustulosis: an Autoimmune Disease Precipitated by Smoking?

Article

Full-text available

Feb 2002

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.

Women with Palmoplantar Pustulosis Have Disturbed Calcium Homeostasis and a High Prevalence of Diabetes Mellitus and Psychiatric Disorders: A Case-Control Study

Article

Full-text available

Jan 2005

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.

Genetic análisis of PSORS1 distinguishes guttate psoriasis and palmoplantar pustulosis

Article

Jan 2003

Acrodermatitis Continua vel perstans (Dermatitis Repens) and Psoriasis Pustulosa

Article

Jul 2006
BRIT J DERMATOL

H. W. BARBER

Recalcitrant Pustular Psoriasis Successfully Treated With Adalimumab

Article

Mar 2011
PEDIATR DERMATOL

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.

Recalcitrant Palmoplantar Pustular Psoriasis Treated with Etanercept

Article

Mar 2011
PEDIATR DERMATOL

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.

Treatment of palmoplantar psoriasis with infliximab: A randomized, double-blind placebo-controlled study

Article

Feb 2011
J EUR ACAD DERMATOL

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.

Sub-polar lepromatous leprosy localized to the face

Article

Sep 2010
Dermatol Online J

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 is a disease distinct from psoriasis

Article

Apr 2011
J DERMATOL TREAT

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.

Ustekinumab in the treatment of palmoplantar pustulosis

Article

Nov 2010
BRIT J DERMATOL

What are the best outcome measures for assessing plaque psoriasis severity? A systematic review of the literature

Article

Apr 2010
J EUR ACAD DERMATOL

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.

Can we really separate palmoplantar pustulosis from psoriasis?

Article

Mar 2010
J EUR ACAD DERMATOL

A retrospective Analysis of treatment responses of palmoplantar psoriasis in 114 patients

Article

Mar 2009
J EUR ACAD DERMATOL

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: A phenotypical and clinical review with introduction of a new quality-of-life assessment tool

Article

Jul 2009

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.

Extra-palmoplantar lesions associated with palmoplantar pustulosis

Article

Jun 2009
J EUR ACAD DERMATOL

T Yamamoto

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.

Association between palmoplantar pustulosis and cigarette smoking in Brazil: A case-control study

Article

May 2009
J EUR ACAD DERMATOL

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.

Efalizumab for severe palmo-plantar psoriasis: An open-label pilot trial in five patients

Article

Feb 2009
J EUR ACAD DERMATOL

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.

Palmoplantar Pustular Lesions during Ovulation Inducement Therapy: New Insight into the Pathomechanism of Palmoplantar Pustulosis?

Article

Apr 2009
DERMATOLOGY

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Hellgren L & Mobacken H. Pustulosis palmaris et plantaris. Prevalence, clinical observations and prognosis. Acta Derm Venereol51: 284-288

Article

Feb 1971

The spectrum of nail involvement in palmoplantar pustulosis

Article

Jul 1996

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.

Palmoplantar pustulosis: A clinical and immunohistological study

Article

Apr 1998

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.

Expression of nicotinic receptors in the skin of patients with palmoplantar pustulosis

Article

Apr 2002

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.

Genetic Analysis of PSORS1 Distinguishes Guttate Psoriasis and Palmoplantar Pustulosis

Article

May 2003

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: A clinicoepidemiological study. The relationship between tobacco use and thyroid function

Article

Jun 2003

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.

Patients with palmoplantar psoriasis have more physical disability and discomfort than patients with other forms of psoriasis: Implications for clinical practice

Article

Aug 2003
J AM ACAD DERMATOL

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.

Coeliac disease-associated antibodies correlate with psoriasis activity

Article

Nov 2004

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.

Interventions for chronic palmoplantar pustulosis

Article

Feb 2006

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.

Pustulosis palmaris et plantaris with prominent hyperkeratosis of the soles

Article

Jan 2007

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.

A classification of psoriasis vulgaris according to phenotype

Article

Mar 2007

For nearly 200 years it has been appreciated that plaque psoriasis consists of a number of distinct clinical phenotypes. However, a reliable and simple stratification of clinical presentation of psoriasis is lacking. In the era of immunogenetic association studies and an advanced understanding of the pathomechanisms of psoriasis it is important that a classification of the disease according to phenotype is readily available. Such a classification would facilitate clinically relevant interpretation of investigational data. A meeting of the International Psoriasis Council produced a consensus on clinical phenotypes of psoriasis equally relevant to clinical practitioners and psoriasis researchers.

Clinical and epidemiological comparison of patients affected by palmoplantar plaque psoriasis and palmoplantar pustulosis: A case series study

Abstract

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