Intraepidermal IgA pustulosis.
Since 1979, 29 patients with intraepidermal IgA detected by direct immunofluorescence have been reported. A review shows that they have a cutaneous disease clinically similar to subcorneal pustular dermatosis, or Sneddon-Wilkinson disease. The pustules may be subcorneal or intraepidermal. IgA deposits are usually found on the intercellular substance of the epidermis, although a subcorneal linear pattern has also been described. Circulating IgA class antiepidermal autoantibodies may be present. The disease usually responds to dapsone. In six cases, a monoclonal IgA gammopathy was present. Although little is known about the pathogenesis of this condition, we believe that it represents a distinct entity among the group of the neutrophilic dermatoses. Various diagnostic terms have been used; we propose intraepidermal IgA pustulosis.
Wallach
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Department of Dermatology, Hôpital Saint-Louis, Paris, France.
D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D003622:Dapsone / Q000627:therapeutic use; D005260:Female; D005455:Fluorescent Antibody Technique; D006801:Humans; D007070:Immunoglobulin A / Q000032:analysis*; D008297:Male; D008875:Middle Aged; D012872:Skin Diseases, Vesiculobullous / Q000188:drug therapy / Q000276:immunology*
D007070:Immunoglobulin A; D003622:Dapsone