Paraneoplastic pemphigus



Paraneoplastic pemphigus

Paraneoplastic pemphigus is a variant of pemphigus, quite distinct from p. vulgaris and p. foliaceus. Paraneoplastic pemphigus may be associated with a variety of tumors, such as B-cell lymphoproliferative disorders and hematopoietic malignancies, Castleman's disease, Waldenström's macroglobulinemia, thymoma (occasionally with myasthenia gravis), hodgkin's lymphoma, carcinomas (e.g., carcinoma of bronchus, pancreas, liver, uterus, breast, and liver), and sarcomas (including dendritic follicular cell sarcoma, round cell liposarcoma, leiomyosarcoma, and inflammatory myofibroblastic tumor). We have seen an exceptional association with systemic mastocytosis. Lymphoma is most often the coexistent neoplasm. 1 In a case of a patient with non-hodgkin's lymphoma, the disease developed only after six cycles of fludarabine, raising the possibility of an association with the medication. In a further exceptional case, a patient presented with a disease fulfilling the diagnosis of paraneoplastic pemphigus by histology, immunoblotting, and immunoprecipitation. however, no neoplasm was found in 8 years of follow-up.

Source: P. McKee, J. Calonje - McKee's Pathology of the Skin (Elsevier)

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