Paraneoplastic pemphigus associated with pelvic inflammatory fibrosarcoma: a case report.
A 36-year-old African-American woman presented with an extensive stomatitis and pigmented cutaneous macules on the neck, axillae and hands. Subsequently she developed violaceus papules on the dorsa of the hands, histologically consistent with an interface dermatitis. After 18 months of progressive disease, paraneoplastic pemphigus was suspected and a search for an underlying neoplasm was initiated. An exploratory laparotomy revealed a pelvic mass and the histologic examination showed an inflammatory fibrosarcoma. The evidence of acantholysis on new cutaneous lesions and the positivity of indirect immunofluorescence with rodent urinary bladder epithelium reinforced the diagnostic criteria for paraneoplastic pemphigus, which is confirmed by the identification of strong protein bands at 210, 190 and 170 kd by immunoprecipitation. Paraneoplastic pemphigus should be considered when investigating atypical mucocutaneous manifestations of pemphigus vulgaris and lichen planus. Diagnostic screening for paraneoplastic pemphigus and a search for an underlying tumor should be performed.
Department of Dermatology, University of São Paulo, São Paulo, Brazil.
Claudia G
CG
Santi
Rosalba Sánchez
RS
Flores
Martha Martínez
MM
Medina
Celina W
CW
Maruta
Valeria
V
Aoki
Grant
G
Anhalt
Evandro A
EA
Rivitti
D000328:Adult; D001741:Black or African American; D001707:Biopsy, Needle; D005260:Female; D005354:Fibrosarcoma / Q000175:diagnosis / Q000473:pathology* / Q000601:surgery; D005500:Follow-Up Studies; D006801:Humans; D007150:Immunohistochemistry; D007813:Laparotomy / Q000379:methods; D010257:Paraneoplastic Syndromes / Q000175:diagnosis / Q000473:pathology* / Q000601:surgery; D010386:Pelvic Neoplasms / Q000175:diagnosis / Q000473:pathology* / Q000601:surgery; D010392:Pemphigus / Q000175:diagnosis / Q000473:pathology* / Q000601:surgery; D018570:Risk Assessment; D016896:Treatment Outcome