Coexistent pemphigus vulgaris and Paget's disease of the nipple. An immunohistochemical study.

We report the coexistence of pemphigus vulgaris and Paget's disease of the nipple in a patient with a documented history of pemphigus and a 2-year complaint of an eczematous breast lesion. A biopsy taken from the lesion showed the characteristic histologic features of both pemphigus and Paget's disease of the nipple. Immunohistochemical technics revealed intercellular IgG deposits characteristic of pemphigus and intracellular staining with antibody to epithelial membrane antigen in the cytoplasm of Paget's cells. This report is the first describing the coexistence of these two distinct disease entities in one tissue sample. This case underscores the necessity of early biopsy of recalcitrant nipple lesions even in the presence of another, well-documented skin disorder.