PMID: 18713592Aug 21, 2008Paper

Paraneoplastic pemphigus associated with non-Hodgkin lymphoma

Dermatology Online Journal
Mariana Dias BatistaFernando Augusto de Almeida

Abstract

We report a case of a 55-year-old man who, after a 6-month history of enlargement of cervical lymph nodes, presented with multiple painful ulcerations of the oral mucosa and lips and multiple skin erosions on the trunk, back, extremities, and genitals. A lymph node biopsy was performed and revealed diffuse peripheral B-cell non-Hodgkin lymphoma. Skin biopsy revealed an acantholytic blister in the epidermis. Direct immunofluorescence showed IgG deposition in the intercellular spaces of the epidermis and linear C3 deposition in the basement-membrane zone. The indirect immunofluorescence test on rat urinary bladder epithelium was positive with a 1:320 titre. Paraneoplastic pemphigus was diagnosed based on these findings; treatment was started with cyclophosphamide, doxorubicin, vincristin and prednisone. The patient's response to treatment was poor and he developed several complications and died 2 months after diagnosis.

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