Subcorneal Pustular Dermatosis in Childhood: A Case Report and Review of the Literature

Abstract

Subcorneal pustular dermatosis (SCPD, also known as Sneddon-Wilkinson disease) is a rare, benign, chronic, sterile pustular eruption which usually develops in middle-age or elderly women; it is rarely seen in childhood and adolescence. The primary lesions are pea-sized pustules classically described as half-pustular, half-clear flaccid blisters. Histologically the most important feature is a subcorneal accumulation of neutrophils with the absence of spongiosis or acantholysis, although acantholysis may be reported in older lesions. In this paper we present the case of a 7-year-old boy diagnosed with SCPD based on the characteristic clinical and histological features. Dapsone has been successfully used in the treatment of the disease.

Subcorneal Pustular Dermatosis: A Review of 30 Years of Progress

“…It is four times more common in women than in men [2,5] and is extremely rare in children [6]. Predilection for a certain race has not been discussed [1], nor has there been a geographically favored region in terms of its incidence or prevalence [7].…”

“…In SPD-type IgA pemphigus, IgA is directed exclusively against desmocollin 1, a desmosomal component of keratinocytes [11,13,60]. DIF studies of perilesional skin demonstrate IgA deposits concentrated in the upper epidermis [2,5,13,14]. A similar result is seen with IIF using patient sera and substrates such as healthy human skin, monkey esophagus, or other epithelia [13,61]; however, about 50 % of IIF studies in SPD-type IgA pemphigus are negative [13,56].…”

“…DIF reveals a granular subepidermal IgA deposition in the tips of the dermal papillae. If it is associated with celiac disease, there is immunoglobulin haphazardly deposited at the jejunum [2]. The target antigen is tissue transglutaminase [13].…”

“…It commonly presents in middle-aged women as a relapsing pustular skin disorder, similar to a variety of erythematous and pustular diseases. Although it is typically seen in adults, there have been a few cases found in children [2][3][4]. Its diagnosis is made on the basis of characteristic clinical and histopathologic findings.…”

A Review and Proposed Approach to the Neutrophilic Dermatoses of Childhood

“…They may also rupture, leaving crusting. It is most commonly reported in adults, but there have been 16 cases reported in children as reviewed by Scalvenzi et al . First‐line treatment is dapsone.…”