Abstract

Pemphigus vulgaris lesions have a tendency to localize around natural body orifices. The aim here was to analyze the involvement of nail apparatus in pemphigus vulgaris. Sixty seven ethnic Poles suffering from pemphigus vulgaris on photographic files archiving initial presentation were retrospectively evaluated. Pemphigus vulgaris was diagnosed using combination of clinical data, H+E histology, direct immunofluorescence of plucked scalp hair and/or perilesional tissue also for IgG1 and IgG4 deposits evaluation, indirect immunofluorescence on mosaic substrate and/or monkey esophagus, mono-analyte ELISA with desmoglein 1/3 or multi-analyte ELISA. The nail apparatus involvement was found in 9 of 67 patients (13.4%; 3 females and 6 males). Periungual fingernail lesions were found in 6 patients (2 females, 4 males), whereas periungual toenail lesions in just 3 patients (1 female, 2 males). Our patients nail apparatus changes included, by order of frequency, paronychia, nail discoloration, onychorrhexis, Beau lines, periungual hemorrhages, onychomadesis, cross-ridging, onycholysis, and trachyonychia. The average time between the onset, as recalled by patients, and the diagnosis of pemphigus vulgaris with direct immunofluorescence was not statistically different in PV patients with and without nail apparatus lesions. In this article the molecular and immunological rationale for of periungual involvement is discussed. Our single-center study suggests that nail apparatus involvement is infrequent in pemphigus vulgaris in ethnic Poles. Due to the fact that nail apparatus lesions in pemphigus vulgaris may clinically resemble onychomycosis, giving the proper diagnosis can be difficult particularly when other lesions are overlooked or misinterpreted.

Highlights

  • Pemphigus vulgaris (PV) is the most common constitute of pemphigus group of autoimmune blistering dermatoses (ABD)

  • In 51 cases of pemphigus vulgaris (PV) (9 with nail apparatus involvement, 42 without nail involvement) we compared the time between the onset of the disease and the diagnosis made with direct immunofluorescence study (DIF) with Mann-Whitney U test with correction for continuity (CI 0.95)

  • The comparison of the wtD in mucocutaneous PV (mcPV) and mucosal dominant PV (mdPV) subtypes was assessed with MannWhitney U test with correction for continuity (CI 0.95), whereas the comparison of wtD in mcPV, mdPV, cutaneous PV (cPV) subtypes was evaluated with Kruskal-Wallis test (CI 0.95)

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Summary

Introduction

Pemphigus vulgaris (PV) is the most common constitute of pemphigus group of autoimmune blistering dermatoses (ABD). In mucocutaneous PV (mcPV), pathological autoimmunity targets desmoglein 3

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