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Background:
Pemphigus is an autoimmune bullous disease affecting the skin and mucous membranes. Associated nail involvement is underestimated and is characterized by a variety of clinical manifestations. Our aim was to describe the clinical aspects of nail involvement during pemphigus.
Patients and methods:
A retrospective study was conducted of...
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Background:
Pemphigus is an autoimmune blistering disease of the skin and mucous membranes caused by autoantibodies against desmoglein 1 (Dsg1) and desmoglein 3 (Dsg3). Pemphigus vulgaris (PV) is the most common form of pemphigus. The aim of this study was to assess the correlation between the levels of anti-desmoglein 1 and 3 autoantibodies and t...
Background:
Desmoglein (Dsg) 1 and 3 are the 2 major autoantigens in pemphigus foliaceus (PF) and pemphigus vulgaris (PV).
Aim:
We aimed to determine anti-Dsg1 and 3 Abs'usefulness in the diagnosis of pemphigus and to assess the correlation of these antibodies (Abs) with clinical phenotype and disease activity in south Tunisian patients.
Method...
Impetigo is a common skin infection. It is highly infectious; caused by gram-positive Streptococcus pyrogens and Staphylococcus; affects mostly children because of weak immunity and frequent contact with others carrying the infection. Most impetigo infections resolve themselves without any use of antibiotics. But in case of children, topical antibi...
Citations
... Pemphigus vulgaris (PV) is an autoimmune vesiculobullous disease that causes blistering of the skin and mucous membranes [1]. Nail involvement is uncommon and underreported [2]. We describe a case of PV in a 20-year-old male who presented with onychodystrophy, corresponding with the severity of his oral and cutaneous lesions and improving with PV treatment. ...
... We report the case of a 20-year-old patient with PV who exhibited nail changes affecting his fingernails, corresponding to a flare in his PV. Nail changes associated with PV are either uncommon or underreported [2]. In a retrospective study conducted in the United States analyzing nail changes in patients with pemphigus (N = 141 patients), not limited to PV, 26% of patients had non-fungal nail changes that correlated with pemphigus disease severity, the most common being pachyonychia (25%), paronychia (25%), and onycholysis (21.5%) [2]. ...
... Nail changes associated with PV are either uncommon or underreported [2]. In a retrospective study conducted in the United States analyzing nail changes in patients with pemphigus (N = 141 patients), not limited to PV, 26% of patients had non-fungal nail changes that correlated with pemphigus disease severity, the most common being pachyonychia (25%), paronychia (25%), and onycholysis (21.5%) [2]. In PV patients, specifically, nail changes were present in 47% of cases. ...
Pemphigus vulgaris (PV) mainly causes blistering of the skin and mucous membranes, with nail unit involvement being rare. Nail involvement may serve as an indicator of disease severity. We present a case of a 20-year-old male with PV who had both cutaneous and nail findings, with nail changes corresponding with disease severity. The patient with biopsy-confirmed PV, on prednisone and mycophenolate, presented to the emergency department with an acute flare of PV and severe mandibular pain and lymphadenopathy. At follow-up in our outpatient department, the physical examination was significant for onychomadesis and onycholysis of the fingernails. Prednisone and mycophenolate dosages were increased, and rituximab infusions were initiated. Bullae and mucosal lesions resolved on the follow-up, and nail changes improved. This case appends an unusual perspective to the limited literature on PV-associated nail changes, especially in younger patients. It advocates for meticulous history taking and physical examination and supports a correlation between nail symptoms and PV disease severity.
... Among mucous membranes, the most commonly involved is the oral mucosa. Although nail involvement is occasionally detected in PV patients, it is underappreciated and viewed as a sign of the severity of PV (2,3). Behçet's disease (BD) is a complex multisystem disease characterised by the presence of oral ulcers, genital ulcers, synovitis, erythema nodosum, folliculitis or posterior uveitis (4). ...
... Differential diagnoses of SADA include subungual warty dyskeratoma, Darier disease, or pemphigus with nail involvement. [7,8] They share a similar pathology with suprabasilar acantholysis and dyskeratosis. In subungual warty dyskeratoma and Darier's disease, similar clinical appearances, including longitudinal erythronychia, leukonychia, and distal subungual hyperkeratosis, are reported. ...
... In subungual warty dyskeratoma and Darier's disease, similar clinical appearances, including longitudinal erythronychia, leukonychia, and distal subungual hyperkeratosis, are reported. [7,8] Paronychia and onychomadesis have been found in pemphigus with nail involvement. [8] In conclusion, SADA is a rare nail lesion that may mimic other inflammatory or neoplastic nail diseases. ...
... [7,8] Paronychia and onychomadesis have been found in pemphigus with nail involvement. [8] In conclusion, SADA is a rare nail lesion that may mimic other inflammatory or neoplastic nail diseases. Pathology is essential to reach the correct diagnosis. ...
... One study suggested that hemorrhagic nail abnormalities might be associated with a poor prognosis in patients with PV (9). The above findings indicate that nail involvement may be a clinical sign correlated with the severity of pemphigus and BP (4,(8)(9)(10)(11)(12). ...
... Nail characteristics were classified according to each type of nail change (4,8,11,13). Disease severity was evaluated by the autoimmune bullous skin disorder intensity score (ABSIS) and antibody titers. ...
... Nail involvement in AIBDs occurs either before or in conjunction with a flare of pre-existing disease, and is rarely a part of the initial presentation (8,11,12). It is consistent with our observation that 96.6% (142/147) of nail involvement in patients with AIBDs occurs after the onset of cutaneous or mucosae lesions. ...
Common autoimmune bullous diseases (AIBDs) include pemphigus and bullous pemphigoid (BP), which are primarily caused by IgG autoantibodies against the structural proteins of desmosomes at the cell–cell junction and hemidesmosomes at the epidermal–dermal junction. Few studies have assessed nail changes in patients with pemphigus or BP. In the present study, we collected the clinical data of 191 patients with AIBDs (108 patients with pemphigus and 83 patients with BP) and 200 control subjects. Nail changes were observed in 77.0% (147/191), 77.8% (84/108), and 75.9% (63/83) of patients with AIBDs, pemphigus, and BP, respectively, and 14.5% (29/200) of control subjects. Beau's lines and paronychia were the most common nail involvement, observed in 22.5% (43/191) and 22.5% (43/191) of patients with AIBDs, 25.0% (27/108) and 25.9% (28/108) of patients with pemphigus, 19.3% (16/83) and 18.1% (15/83) of patients with BP, respectively. The autoimmune bullous skin disorder intensity score (ABSIS) and the onset time of patients with pemphigus or BP with nail changes were different. Onychomycosis accounted for 21.5% (41/191) of all patients with AIBDs. The ABSIS was correlated with nail involvement in patients with BP ( r = 0.46, p < 0.001), and weakly correlated with nail involvement in patients with AIBDs ( r = 0.37, p < 0.001), pemphigus ( r = 0.29, p = 0.009), and pemphigus vulgaris (PV; r = 0.35, p = 0.008). No correlation was observed between nail involvement and disease antibody titers. In conclusion, nail changes are frequently observed in patients with pemphigus and BP. The type and onset time of nail changes may indicate the severity of pemphigus and BP, which warrants the attention of dermatologists.
... Complete nail loss has not previously Skin Appendage Disord 2022;8:504-507 DOI: 10.1159/000525462 been reported. It is thought that nail involvement correlates with disease severity and may be evident before, during, or after other symptoms of the disease [4]. Here, we report a case of PV with unusual presentation. ...
... Reported prevalence of nail involvement in PV patients includes 13% (Poland) [2], 80% (India) [5], 32% (Iran) [6], and 47% (USA) [3]. A more recent study in the USA in 2019 with 141 pemphigus patients reported nail involvement prevalence of 43% (60/141) but only 26% (37/141) when fungal origin was eliminated [4]. However, this study included all pemphigus patients, not PV patients exclusively. ...
... However, this study included all pemphigus patients, not PV patients exclusively. Though some studies report no association between severity of disease and nail involvement, the aforementioned study found a significant correlation between the presence of ungual involvement with severity of pemphigus, particularly severe oral disease (p = 0.002) [4]. Therefore, our patient with mild to moderate PV and nail involvement is unique. ...
Introduction:
Symptoms of pemphigus vulgaris (PV) rarely include nail findings. When ungual involvement does occur, the most common manifestations are paronychia and onychomadesis. Onycholysis is very uncommon, and complete nail loss has not been reported in the literature. Ungual involvement is thought to be closely correlated with disease severity, with only severe PV cases demonstrating nail symptoms.
Case presentation:
We report a case of a 34-year-old female presenting with mild to moderate PV yet severe onycholysis of her first toe leading to secondary nail loss in conjunction with paronychia of her bilateral thumbs. Oral tofacitinib and rituximab infusions led to strikingly rapid improvement in her nail symptoms.
Discussion/conclusion:
This case illustrates the importance of a thorough history and physical exam, as a primary complaint of nail symptoms in these patients may lead to unrecognized PV by even the most skilled practitioners. Tofacitinib and rituximab in combination may rapidly improve nail involvement.
b> Background: There are few studies, mainly case reports, on the involvement of the nail unit in autoimmune bullous disorders. Summary: Nail involvement in autoimmune bullous disorders is a significant clinical phenomenon, marked by a range of manifestations, most often not presenting with blisters like on the skin but rather with alterations of the nail unit such as paronychia, onychomadesis, or onycholysis. This involvement is particularly notable due to the unique immunological features of the nail unit, including the expression of various antigens and the presence of Langerhans cells. Conditions like pemphigus vulgaris, bullous pemphigoid, epidermolysis bullosa acquisita, linear IgA disease, and bullous systemic lupus erythematosus can lead to nail abnormalities. The prevalence of nail manifestations varies according to the disorder, and diagnosis often relies on histopathological and immunofluorescence testing. Nail involvement correlates with disease severity and duration, sometimes serving as a herald sign. Further research is needed to guide therapeutic approaches for nail involvement in autoimmune bullous diseases. Key Messages: Nail involvement in autoimmune bullous nail disorders may be confusing as there are almost never bullae. One should keep the diagnosis in mind when facing an atypical paronychia.
Context:
Autoimmune blistering (AIBD) disorders affect mucosae, skin, and appendages. Appendageal involvement has not been studied extensively to date. However, they are important as nail changes are commonly encountered during a flare-up of the disease.
Aims:
To determine the prevalence and patterns of nail changes in various Immunobullous disorders and to study the relationship between the nail changes and the disease duration and severity.
Settings and design:
A cross-sectional study was conducted at the Department of Dermatology, venerology, and leprosy at a tertiary care center in Mumbai.
Materials and methods:
A cross-sectional study including a total of 74 cases of Immunobullous diseases was conducted and the prevalence of nail changes was determined. The association between the mean number of nail changes and the disease duration and severity was analyzed using ANOVA (Analysis of variance) and unpaired t-test.
Statistical analysis:
The Statistical Package for Social Studies (SPSS) software was used for statistical evaluation.
Results:
We found that the prevalence of nail changes was 91%. There was a significant difference in the mean number of nail changes with respect to the severity grades of mucosal involvement in AIBD (P value < 0.05). There was no significant difference in the mean number of nail changes with respect to the severity grades of Pemphigus vulgaris (PV), Pemphigus foliaceous (PF), subepidermal blistering diseases, and the duration of AIBD.
Conclusions:
Nails are frequently affected in AIBD. The number of nail changes is related to the severity of mucosal disease but not to duration.
Introduction
Les pemphigus est un groupe de pathologies auto-immunes, qui touche principalement la peau et les muqueuses. Les ongles sont le site le plus fréquemment impliqué au cours des pemphigus après les manifestations cutanéomuqueuses. L’atteinte unguéale peut être le seul signe initial évocateur. Les manifestations cliniques les plus courantes comprennent la paronychie chronique et l’onychomadèse.
Matériel et méthodes
Nous avons mené une étude rétrospective entre 2014 et 2021 incluant tous les patients hospitalisés pour pemphigus dans le service de dermatologie du centre hospitalier universitaire Mohammed VI d’Oujda. Le diagnostic de pemphigus reposait sur des données cliniques, immunologiques et histologiques.
Résultats
Pendant une période de 7 ans, 42 cas de pemphigus ont été colligés. Parmi ceux-ci, 28 patients avaient une atteinte unguéale. Après avoir éliminé l’onychomycose, nous avons inclus 16 patients (37 %) dans notre étude. Il y avait 9 hommes et 7 femmes. L’âge moyen était de 56,8 ans. L’atteinte unguéale était trouvée dans tous les types de pemphigus, ainsi il a été recensé 62 % des cas au cours du pemphigus vulgaire, 19 % au cours du pemphigus superficiel, 13 % au cours du pemphigus végétant et 6 % au cours du pemphigus paranéoplasique. L’atteinte unguéale était concomitante aux lésions cutanéomuqueuses chez 19 % des cas, inaugurale chez 62 % des cas et tardive chez 19 % des cas. L’atteinte des ongles des orteils était notée dans 61 % des cas et celle des doigts dans 39 % des cas. Les altérations unguéales les plus fréquentes étaient l’onycholyse (30 %), la paronychie chronique (15 %), les lignes de Beau, la trachyonychie, l’hyperkératose sous unguéale et le ptérygion (10 % chacune), l’onychomadèse, l’onychorrhexis et la dystrophie unguéale (5 % chacune). La présence d’une atteinte unguéale sévère était associée à une durée d’évolution plus longue du pemphigus avant et après le diagnostic.
Discussion
Le diagnostic de pemphigus est basé sur les manifestations cliniques et confirmé par des tests histologiques et immunologiques. Ils se caractérisent par des bulles intra-épidermiques avec une acantholyse suprabasale et des auto-anticorps qui sont exprimés également dans le pli unguéal proximal, la matrice unguéale et l’hyponychium. L’atteinte de l’appareil unguéal peut annoncer la récurrence et l’exacerbation du pemphigus. Les manifestations unguéales sont divers et comprennent principalement la paronychie, l’onychomadèse, l’onycholyse, les lignes de Beau, la trachyonychie, l’onychorrhexis, l’hyperkératose sous-unguéale, le ptérygion et la dystrophie unguéale. Les résultats de notre étude sont compatibles avec les données retrouvées dans la littérature concernant, la fréquence de la localisation unguéale ; la survenue plus fréquente au cours du pemphigus vulgaire et l’aspect clinique.
BACKGROUND Pemphigus is a group of rare, life-threatening autoimmune bullous diseases of the skin and mucosa which result in intraepidermal blistering. Associated autoimmune conditions and the extensive mucocutaneous detachment impair the quality of life. Immunosuppressive treatment adds to the morbidity in patients. We wanted to assess the clinical presentation of pemphigus among the study subjects along with the outcome of pemphigus patients with the extent of the disease and results of pus culture. METHODS Retrospective analysis of case records of patients with pemphigus in a tertiary care hospital during a period of 3 years was done in 2015. Demographic data, clinical findings, underlying medical disorders, details of deaths, investigation reports and treatment details were recorded. RESULTS Common age group affected was 51-60 years (31.5 %) among a total of 54 pemphigus patients. There were 26 males and 28 (51. 9 %) females and in majority of the patients (44.4 %) duration was less than 6 months. Out of 47 patients, oral mucosal involvement was grade 1 in 20 (37 %) cases, grade 2 in 12 (22.2 %) and in 15 (27.8 %) patients it was grade 3.Histopathological examination showed suprabasal cleft in 82.1 %, subcorneal blister in 5.7 %, acantholytic cells in 48.6 % and row of tombstone appearance in 9.4 %. Out of 36 patients, DIF test reported intercellular IgG in 65 % and C3 in 50 % .Diabetes was found in 14 (25.9 %), candidiasis in 12 (22.2 %), thyroid disease in 6 (11.1 %) and hypertension in 3 patients. Three patients died. Among 10 patients with more than 30 % body surface area involvement, 1 (10 %) patient died. Two (13 %) deaths occurred among 15 patients in whom pus C&S was positive. CONCLUSIONS Pemphigus patients were of slightly higher (51 - 70 years) age group and the 3 patients died were females. Autoimmune disorders like diabetes and thyroid disorders were found among many patients and their family members. Infection could be an important cause of death, but no statistically significant association was found in the present study. KEYWORDS Pemphigus, Steroids, Comorbidity, Deaths
