Paraneoplastic pemphigus associated with non-Hodgkin lymphoma.
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.
Batista
Mariana Dias
MD
Department of Dermatology, Federal University of Sao Paulo - UNIFESP, Brazil. mariana_db@uol.com.br
Takano
Daniela
D
Lopes
Renato Delascio
RD
Enokihara
Milvia M S S
MM
Michalany
Nilceo Schwery
NS
de Almeida
Fernando Augusto
FA
D000051:Acantholysis / Q000209:etiology / Q000473:pathology; D000818:Animals; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects; D001415:Back / Q000473:pathology; D003520:Cyclophosphamide / Q000009:adverse effects; D004317:Doxorubicin / Q000009:adverse effects; D017809:Fatal Outcome; D019085:Fluorescent Antibody Technique, Direct / Q000379:methods; D006801:Humans; D008228:Lymphoma, Non-Hodgkin / Q000150:complications*; D008297:Male; D008875:Middle Aged; D009061:Mouth Mucosa / Q000473:pathology; D010257:Paraneoplastic Syndromes / Q000188:drug therapy / Q000209:etiology* / Q000378:metabolism / Q000473:pathology; D010392:Pemphigus / Q000188:drug therapy / Q000209:etiology* / Q000378:metabolism / Q000473:pathology; D011241:Prednisone / Q000009:adverse effects; D051381:Rats; D012867:Skin / Q000473:pathology; D014750:Vincristine / Q000009:adverse effects
D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone