Extramammary Paget disease
See also in: AnogenitalAlerts and Notices
Synopsis

The condition is most often seen in elderly White women, but males are more likely to be diagnosed with this condition in Japan.
Clinically, lesions most commonly appear on the vulva in women and in the perianal area in men. Lesions are often banal in appearance and similar to the lesions of eczematous dermatoses, leading to delays in diagnosis. Symptoms may be minimal to none; sometimes, there is itch, pain, or burning. Presentation is typically of a red, sharply demarcated plaque that expands slowly. Areas within the plaque are marked by erosions and white scale, leading to the appearance of "strawberries and cream." EMPD is thought to invade and metastasize via lymphatics.
Codes
ICD10CM:C44.99 – Other specified malignant neoplasm of skin, unspecified
SNOMEDCT:
71447003 – Paget's disease, extramammary (except Paget's disease of bone)
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Differential Diagnosis & Pitfalls
Clinically, the plaques of EMPD resemble those of eczematous dermatoses. However, the plaques of eczematous diseases lack sharp borders. Furthermore, EMPD often has hydrated scale and erosions, giving a "strawberries and cream" appearance. Clinical suspicion should become elevated when clinical response to treatment of presumed eczematous dermatitis is not noted.- Allergic / irritant contact dermatitis
- Seborrheic dermatitis
- Psoriasis
- Lichen simplex chronicus
- Bowen disease
- Basal cell carcinoma
- Candidiasis
- Tinea cruris
- Intertrigo
- Hailey-Hailey disease
- Glucagonoma syndrome
- Lichen sclerosus
- Lichen planus
- Erythroplasia of Queyrat
- Pemphigus vegetans
- Melanoma
- Pagetoid dyskeratosis
- Clear cell papulosis
- Langerhans cell histiocytosis
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References
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Last Reviewed:09/11/2018
Last Updated:12/07/2021
Last Updated:12/07/2021
Extramammary Paget disease
See also in: Anogenital