Extramammary Paget's disease physical examination

Common physical examination findings of extramammary Paget’s disease include well demarcated, erythematous or leucoplakic plaques present on the skin, characteristic ‘cake-icing’ appearance of vulval extramammary Paget’s disease, consisting of erythematous changes associated with white islands and bridges of hyperkeratotic epithelium, and lymphadenopathy.

Early skin changes may be subtle and vary according to location. Only slight erythema, crusting, and increased maceration may be noted, initially. However, pruritus commonly leads to prominent lichenification and excoriations. The most commonly affected site is the vulva, followed by perineal, perianal, scrotal and penile skin. Less commonly, the buttocks, axilla, thighs, eyelids and external auditory canal may be affected.^[1][2]

A detailed review of systems and physical examination should be performed in all patients with extramammary Paget’s disease. The examination should include the following:^[1][3]

• Full skin examination
• Palpation of all lymph nodes
• Rectal examination

• Well-demarcated, erythematous or leucoplakic plaques are present.
• May appear as chronic intertrigo or presumed tinea cruris

• The affected skin may appear eczematous, papillomatous, lichenified, leucokeratotic, or ulcerated
• Whitish discharge may be present
• Characteristic ‘cake-icing’ appearance of vulval extramammary Paget’s disease, consisting of erythematous changes associated with white islands and bridges of hyperkeratotic epithelium
• Inguinal lymphadenopathy may present
• A palpable mass with or without lymphadenopathy raises suspicion of invasive disease

• Lymphadenopathy may be present

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  Extramammary paget’s disease. With permission from Dermatology Atlas.^[4]

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