Track

Case Reports

Abstract

Extramammary Paget’s disease (EMPD) and verruciform xanthoma (VX) are two distinct, uncommon entities with unique pathogeneses and clinical behavior. EMPD is a cutaneous malignant neoplasm characterized by Paget cells in the epithelium, which can arise in the skin or reflect extension to the skin from a deeper adnexal or other carcinoma, depending in part on the site. Immunostaining is usually performed for classification. Surgical excision is usually the definitive treatment. VX are benign growths most commonly occurring in the oral mucosa, with the remaining minority distributed among genital areas and extremities. These tend to behave indolently. Both diseases are uncommonly reported to arise concurrently with another skin proliferation. We present a rarely reported case of concurrent scrotal EMPD and VX in a 94-year-old male. Only two other cases have been documented. Initial presentation included pruritus and erythema with scale of the scrotum. Biopsy revealed intraepidermal cells with atypical nuclei and abundant vacuolated cytoplasm displaying pagetoid scatter. These labeled positively with CK7 and EMA and were CK20- and SOX-10-negative. The background skin was pedunculated, with epidermal hyperplasia and foamy macrophages in widened dermal papillae, and parakeratosis, characteristic of VX. Clinically, early EMPD often presents nonspecifically and indolently as in this case which can lead to delay in diagnosis. This case highlights the prudence of retaining EMPD in the differential diagnosis of longstanding anogenital lesions that fail to respond to typical treatment, and a rare collision of two unusual entities.