Extramammary Paget Disease

Extramammary Paget Disease is a rare skin condition that mainly affects areas with many sweat glands, such as the genital, anal, and armpit regions. It involves abnormal growth of cells in the skin's top layer, which can cause red, scaly, and itchy patches that may look like eczema or other skin problems. This disease primarily affects the skin and underlying tissues and can sometimes be linked to cancers in nearby organs. The main health impact is persistent skin irritation and the potential for the disease to spread or be associated with internal malignancies. Diagnosis often requires a skin biopsy to identify the abnormal cells. Treatment usually involves surgery or other methods to remove the affected skin. Early detection is important to manage symptoms and prevent complications.

Extramammary Paget Disease (EMPD) is a rare intraepithelial adenocarcinoma characterized by the presence of malignant Paget cells within the epidermis of apocrine gland–rich areas such as the vulva, perianal region, scrotum, and axilla. It arises either as a primary cutaneous neoplasm or secondary to an underlying malignancy, often from the genitourinary or gastrointestinal tract. The disease manifests as a chronic, erythematous, and eczematous plaque with possible ulceration and pruritus. Histologically, EMPD shows large pale-staining Paget cells with prominent nuclei scattered singly or in clusters within the epidermis. Immunohistochemical staining typically reveals positivity for cytokeratin 7 (CK7) and gross cystic disease fluid protein-15 (GCDFP-15), aiding in differentiation from other dermatoses. EMPD is clinically significant due to its potential for local invasion, recurrence, and association with internal malignancies, necessitating thorough evaluation and management.