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

✍ Scribed by H. Yasuda; H. Kobayashi; T. Hashimoto; K. Itoh; M. Yamane; J. Nakamura

• Publisher: John Wiley and Sons
• Year: 2000
• Tongue: English
• Weight: 431 KB
• Volume: 143
• Category: Article
• ISSN: 0007-0963
• DOI: 10.1046/j.1365-2133.2000.03604.x

✦ Synopsis

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.