IgA Pemphigus foliaceus: A Case Report

Abstract

An 84-year-old male with a 4-week history of vesiculobullous eruptions was diagnosed clinically and pathologically to suffer from pemphigus foliaceus. Amazingly, direct immunofluorescence demonstrated intercellular IgA deposits in the whole epidermis. The patient did not respond to a regimen of fluocortolone and azathioprine but rapidly improved under dapsone. The few reported dermatoses with intraepidermal IgA deposits do not form a homogeneous group. In the following, their spectrum will be outlined. It is important to distinguish these dermatoses from IgG pemphigus, since they require a different therapy.

Azathioprine in the Treatment of Autoimmune Blistering Diseases

“…Dapsone is usually the firstline treatment. 21 For pemphigus vegetans, azathioprine seems to work in individual cases. 22 For treatment of refractory patients, disease control has been reported under the corticosteroid/azathioprine regimen using additionally photopheresis or retinoids.…”

Intraepidermal Neutrophilic IgA Dermatosis: Pemphigus-Like IgA Deposits

“…Zillikens et al [14] have proposed a classification of der matoses with intraepidermal IgA deposits into three sub groups following the localization of IgA deposits and pus tules in the epidermis.…”

Intra-Epidermal Immunoglobulin A Pustulosis

“…[3], This dis ease is probably not very rare. An additional case, described by Zillikens et al [4], appears in this issue of Dermatológica. 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.…”