Extramammary Paget's disease physical examination

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Kiran Singh, M.D. [2]Simrat Sarai, M.D. [3]

Physical Examination

At clinical examination, EMPD may appear as chronic intertrigo or presumed tinea cruris. It may appear eczematous, and it has usually been present for a long time 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.

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. Lesional progression leads to a unilateral sharply marginated plaque with distinct erythema. Superficial erosion or scaling may develop in mature lesions.

The most frequently affected site is the vulva, followed by perineal, perianal, scrotal and penile skin. Less commonly, the axilla, buttocks, thighs, eyelids and external auditory canal may be affected.9 If EMPD arises at sites relatively free of apocrine glands, it is referred to as ‘ectopic EMPD’. Multifocal Paget's disease has been reported, usually comprising coincident anogenital and axillary disease, or less frequently concurrent mammary and extramammary disease.10

Patients present with well-demarcated, erythematous or leucoplakic plaques. Most cases appear eczematous but others are crusting, scaling, papillomatous, lichenified, leucokeratotic, ulcerated or bleeding.11 Dubreuilh4 was the first to describe the characteristic ‘cake-icing’ appearance of vulval EMPD, consisting of erythematous changes associated with white islands and bridges of hyperkeratotic epithelium. A palpable mass with or without lymphadenopathy raises suspicion of invasive disease.

Pruritus is the most common symptom, occurring in around 70% of patients. Other complaints include burning, irritation, pain, tenderness, bleeding and swelling. The disease is asymptomatic in 10% of patients. The average time interval from the onset of symptoms to diagnosis is two years.5,12,13

Skin

Vulva

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  Extramammary paget's disease. With permission from Dermatology Atlas.

References

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