Extramammary Paget Disease Clinical Presentation

Updated: Jul 21, 2021
  • Author: Richard Harold "Hal" Flowers, IV, MD; Chief Editor: Dirk M Elston, MD  more...
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Presentation

History

The possibility of extramammary Paget disease (EMPD) should be carefully considered in any patient with chronic dermatitis of the groin, vulva, or perianal area that is recalcitrant to treatment. Patients with EMPD usually present with nonresolving eczematous lesions in the groin, genitalia, perineum, or perianal area. [16] The most common symptom of EMPD is intense pruritus; most patients have only pruritus in the affected area and no other symptoms. Pain and bleeding may occur in longer-standing lesions. Burning, tenderness, and edema may also be experienced over the area of involvement.

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Physical Examination

At clinical examination, extramammary Paget disease (EMPD) may appear as chronic intertrigo, eczematous dermatitis, or presumed tinea cruris. Lesions can be nonspecific, making diagnosis challenging. EMPD usually has been present for several years before biopsy is performed to confirm the diagnosis, with a reported median delay of 2 years before proper diagnosis. [1] The genitalia, perineum, axillae, and external auditory canal are rich in apocrine glands; therefore, these are the usual sites of EMPD involvement, which is often multifocal. In males, the penoscrotal and perianal areas are most frequently involved, as is the vulva in women. [1] Rarely, it may occur in the buttock, knee, glans penis, thigh, abdomen, umbilicus, lower anterior chest, and scalp. [17] See the image below.

Classic appearance of extramammary Paget disease ( Classic appearance of extramammary Paget disease (EMPD). Courtesy of Kenneth E Greer, MD.

Early skin changes may be subtle and vary according to location. Initially, only slight erythema, crusting, and increased maceration may be noted. Pruritus commonly leads to prominent excoriations and lichenification. Scattered areas of erosion and white scale can result in a characteristic "strawberries and cream" appearance (see the image below). [1] Lesional progression leads to a unilateral sharply marginated plaque with distinct erythema. Hypopigmentation or hyperpigmentation occurs, and small pale islands are often observed over the lesion.

Classic "strawberries and cream" appearance of ext Classic "strawberries and cream" appearance of extramammary Paget disease (EMPD). Courtesy of Kenneth E Greer, MD.

Hard nodules and regional lymph node enlargement may develop in cases with an underlying carcinoma. "Underpants-pattern erythema" is described in cases of genital EMPD as a result of metastases to the lymphatic system. This pattern starts in the groin and spreads peripherally to areas typically covered by the underwear. This portends a grim prognosis owing to the possibility of rapidly fatal distant metastases. [1]

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