Subcorneal pustular dermatosis and monoclonal IgA

Abstract

A patient with subcorneal pustular dermatosis was found to have a circulating monoclonal IgA kappa immunoglobulin. Direct immunofluorescence studies revealed IgA kappa deposits in the subcorneal zone of the epidermis. Circulating IgA kappa reacting with the subcorneal zone of normal human epidermis was demonstrated by indirect immunofluoresence. It is speculated that IgA deposition might be implicated in the pathogenesis of subcorneal pustular dermatosis.

The Distribution of IgA Pemphigus Antigen in Human Skin and the Role of IgA Anti-Cell Surface Antibodies in the Induction of Intraepidermal Acantholysis

“…We conclude the following: (1) The expression of IgA pemphigus antigen in human skin shows regional vari¬ ability, interindividual variability, and variability in the microscopic distribution within the epidermal cell lay¬ ers. We therefore propose the use of multiple skin sub¬ strates for the routine detection of IgA pemphigus Ab in patients' serum specimens.…”

IgA Pemphigus foliaceus: A Case Report

“…Some cases of each subgroup demonstrated circulating IgA antibodies to intraepidermal structures on indirect immunofluorescence [4,6,8,[10][11][12][13][14], Moreover, in some patients an IgA gam m opathyw asdetcctcd[l, 3,6,12]. In our patient both indirect immunofluorescence and immunoelectrophoresis were normal.…”

“…In 1 case pustules were located subcorncally [12], whereas in the second patient the location of pustules varied with the age of the lesions [13]. Interestingly, this case showed leukocytoclastic vasculitis in early lesions in addition.…”

Intra-Epidermal Immunoglobulin A Pustulosis

“…In the majority of the reported cases, IgA is located on the so-called intercellular substance of the epi dermis. This obviously resembles the pemphigus immu- [5][6][7] may constitute a distinct entity. This is however not a defi nite conclusion.…”

“…It is interesting to note that there is already some heterogeneity among these observations, and this results in some controversy about the denomina tion of the disease and its nosology. Zillikens et al pro pose to classify these patients into three subgroups; IgA pemphigus, as proposed by Beutneret al [3], is itself sub divided into a subcorneal type and an intra-epidermal type; the third subgroup includes 3 cases with subcorneal IgA deposits [5][6][7].…”