Extramammary Paget Disease (EMPD) is a rare, slow-growing cancerous skin condition that primarily affects the outer genital area, perianal region, and other non-mammary regions such as the axilla (armpit) and groin. It is characterized by the presence of abnormal cells, called Paget cells, in the outermost layer of the skin.
EMPD is often associated with an underlying invasive or in situ cancer, such as adenocarcinoma originating in a nearby organ, most commonly the anus, rectum, or urinary tract. However, in a significant number of cases, no underlying malignancy is found.
The disease usually manifests as red, scaly, or eczema-like lesions, which may at times be accompanied by itching, burning, or soreness. Due to its non-specific appearance, EMPD can often be misdiagnosed or mistaken for other dermatological conditions.
Diagnosis of EMPD typically involves a thorough clinical examination, followed by a biopsy of the affected area to analyze tissue samples under a microscope. Additional tests, such as imaging studies or laboratory investigations, may be conducted to determine the presence and extent of an underlying tumor.
Treatment options for EMPD depend on the extent and aggressiveness of the disease, as well as the presence of an underlying malignancy. Surgical excision is often the primary treatment, removing the affected skin and, if necessary, adjacent tissue or lymph nodes. Other therapeutic approaches include topical treatments, radiation therapy, chemotherapy, or immunotherapy.
Following treatment, regular monitoring and follow-up visits are crucial to detect any signs of recurrence or further complications. Overall, the prognosis for patients with EMPD varies depending on the stage of the disease, the presence of an underlying cancer, and individual factors such as
