Abstract
We report a 42-year-old man with paraneoplastic pemphigus who developed severe oral mucosal ulceration and pneumonia. Computed tomography of the abdomen demonstrated a mass, and B cell lymphoma was diagnosed by a celiac lymph node biopsy. The patient's serum was positive for anti-desmoglein 1 and 3 antibodies by enzyme-linked immunosorbent assay. Skin biopsies revealed epidermal vacuolization and suprabasal cleft formation. IgG deposition was observed in intercellular spaces of the epidermis and C3 was also positive along the basement membrane zone on direct immunofluorescence.
An indirect immunofluorescence analysis using normal human skin as its substrate showed antiintracellular IgG antibodies at a titre of 1 in 160, which also reacted with transitional epithelia of rat urinary bladder. The patient's serum immunoprecipitated antigens at 210-kDa envoplakin and 190-kDa periplakin. Prednisolone, cyclosporin and topical steroids were used for mucosal lesion without remarkable improvement. Finally, progressive respiratory failure with clinical features of bronchiolitis obliterans was the cause of death in this case. [Skin Cancer (Japan) 2008; 23: 11-16]
