Ectopic extramammary Paget's disease arising on the cheek.
Overview
abstract
BACKGROUND: Extramammary Paget's disease (EMPD) is a rare neoplasm affecting apocrine gland-bearing skin. Patients usually present with a long-standing unilateral, ill-defined, erythematous or whitish-gray, crusted patch or plaque. This tumor almost never occurs outside of the anogenital or axillary areas. OBJECTIVE: We report the first case of "ectopic" extramammary Paget's neoplasm afflicting the face. METHODS: The excisional specimen from a 67-year-old man was reviewed. Histology and immunohistochemistry studies were performed on the specimen. RESULTS: Histology revealed hyperkeratosis, parakeratosis, acanthosis, and proliferation of cytologically atypical epithelioid cells permeating the epidermis and the cutaneous adnexae. Special stains showed reactivity to cytokeratins (AE1/AE), epithelial membrane antigen, and carcinoembryonic antigen in the atypical cells. There was no evidence of apocrine glands in the area. CONCLUSION: Herein we present the first case of truly "ectopic" extramammary Paget's disease on the face.
