Extramammary Paget’s disease (EPD) is a neoplasm of the anogenital and axillary skin, histologically and clinically similar to Paget’s disease of the breast, and often represents an intraepiderrnal extension of a primary adenocarcinoma of underlying apocrine glands or of the lower gastrointestinal, urinary, or female genital tracts.
Causes of Extramammary Paget’s Disease
The histogenesis of EPD is not uniform. Paget cells in the epidermis may occur as an in situ upward extension of an in situ adenocarcinoma in deeper glands (25%). Alternatively, EPD may have a multifocal primary origin in the epidermis and its related appendages. The primary tumor and paget cells are usually mucus-secreting. Primary tumors in the anorectum can arise within the rectal mucosa or intramuscular glands.
Symptoms of Extramammary Paget’s Disease
At clinical examination, EMPD may appear as chronic intertrigo or presumed tinea cruris. EMPD may appear eczematous, and it has usually been present for a long time before biopsy is performed to confirm the diagnosis.
Initially, only slight erythema, crusting, and increased maceration may be noted.
Diagnosis
Clinical suspicion confirmed by skin biopsy.
Treatment
EPD is usually much larger than is apparent clinically. Surgical excision must be controlled histologically (Mohs’ microscopic surgery). If Paget cells are in dermis and regional lymph nodes are palpable, lymph node dissection may improve prognosis.
References