Extramammary Paget Disease Clinical Presentation

Updated: Apr 18, 2017
  • Author: Blanca Anais Estupiñan; 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. Patients with EMPD usually present with nonresolving eczematous lesions in the groin, genitalia, perineum, or perianal area. [5] 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.

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Physical Examination

At clinical examination, extramammary Paget disease (EMPD) may appear as chronic intertrigo or presumed tinea cruris. It may appear eczematous, and it has usually been present for several years before biopsy is performed to confirm the diagnosis. The genitalia, perineum, axillae, and external auditory canal are rich in apocrine glands; therefore, these are the usual sites of EMPD involvement, which is usually multifocal. In males, the penoscrotal area is most frequently involved, as is the vulva in women.

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. In addition to pruritus, patients may describe a burning sensation or pain over the area of involvement. 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. Superficial erosion or scaling may develop in mature lesions.

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