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Paraneoplastic Pemphigus Associated with Pelvic Inflammatory Fibrosarcoma: A Case Report
Author(s) -
Santi Claudia G.,
Flores Rosalba Sánchez,
Medina Martha Martínez,
Maruta Celina W.,
Aoki Valeria,
Anhalt Grant,
Rivitti Evandro A.
Publication year - 2005
Publication title -
the journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.9
H-Index - 65
eISSN - 1346-8138
pISSN - 0385-2407
DOI - 10.1111/j.1346-8138.2005.tb00893.x
Subject(s) - paraneoplastic pemphigus , mucocutaneous zone , medicine , acantholysis , pemphigus , pathology , dermatology , pemphigus vulgaris , autoantibody , antibody , disease , immunology
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.