Subcorneal pustular dermatosis type of IgA pemphigus: demonstration of autoantibodies to desmocollin-1 and clinical review

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We describe a 40-year-old Japanese man with a 3-year history of vesiculopustular lesions resembling subcorneal pustular dermatosis. Histopathology showed subcorneal pustules containing a few acantholytic cells, and direct immunofluorescence disclosed IgA deposition in the intercellular space of the upper epidermis. Circulating IgA autoantibodies of very low titre were also demonstrated by indirect immunofluorescence. A novel cDNA transfection technique clearly detected IgA autoantibodies reactive with human desmocollin-1. Combined therapy with dapsone and etretinate improved the skin lesions. We review the clinical features of 49 patients in the literature who presented with vesiculopustular lesions and intraepidermal IgA deposition.

Keywords: IgA pemphigus; desmocollin-1; subcorneal pustular dermatosis type

Document Type: Research Article


Affiliations: 1: Department of Dermatology, Sapporo Social Insurance General Hospital, 2-1, 2-Jo, 6-Chome, Atsubetsu Chuo, Atsubetsu, Sapporo 004-8618, Japan 2: Department of Dermatology, Hokkaido University School of Medicine, Sapporo, Japan 3: Department of Dermatology, Kurume University School of Medicine, Fukuoka, Japan 4: Nopporo Dermatology Clinic, Ebetsu, Japan

Publication date: July 1, 2000

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