Paraneoplastic pemphigus: a subset of patients with pemphigus and neoplasia.

Autor: Mehregan, Darius R., Oursler, Judith R., Leiferman, Kristin M., Muller, Sigfrid A., Anhalt, Grant J., Peters, Margot S.
Předmět:
Zdroj: Journal of Cutaneous Pathology; Jun93, Vol. 20 Issue 3, p203-210, 8p
Abstrakt: We reviewed the clinical, histologic, and immunofluorescence features of 20 patients with pemphigus and neoplasia and compared them with a control group of 17 patients with pemphigus without neoplasia. Patients with neoplasia were divided according to clinical, histologic, and immunofluorescence findings into those with paraneoplasitc pemphigus syndrome (12 patients) and those with classic pemphigus vulgaris or pemphigus foliaceus with neoplasia (8 patients). The histologic findings in patients with parancoplastic pemphigus included acantholysis, interface dermatitis, spongiosis, and satellite keratinocyte necrosis. Histologic findings in the 8 patients with classic pemphigus and neoplasia included acantholysis and spongiosis. Direct immunofluorescence in both paraneoplastic pemhigus and pemphigus with neoplasia showed IgG staining of cell-surface proteins (intercellular substance) and deposition of immunoglobulin at the basement membrane zone. Indirect immunofluorescence with rat bladder substrate was used to differentiate paraneoplastic pemphigus from classic pemphigus. Circulating IgG ani-cell-neoplastic pemphigus syndrome; they were absent in 2 patients with pemphigus and neoplasia. Immunoprecipitation of sera from the 4 patients with epithelial staining showed the complex of bands identified in studies of paraneoplastic pemphigus syndrome. We conclude that paraneoplastic pemphigus syndrome has distinct clinical, histologic and immunologic features that differentiate it from classic pemphigus with underlying neoplasia. [ABSTRACT FROM AUTHOR]
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