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Extramammary Paget disease
See also in: Anogenital
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Other Resources UpToDate PubMed

Extramammary Paget disease

See also in: Anogenital
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Contributors: Gaurav Singh MD, MPH, Susan Burgin MD
Other Resources UpToDate PubMed

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Synopsis

Extramammary Paget disease : Genitals, buttocks, perineum, Scaly plaque, Skin erosion, Inguinal region
Extramammary Paget disease (EMPD) is a rare neoplastic condition that usually occurs in the sixth to eighth decades of life. EMPD may be primary and arise as a cutaneous adenocarcinoma, or it may be secondary, due to extension of an underlying contiguous or noncontiguous adenocarcinoma. Approximately 10%-25% of patients have a contiguous malignancy at the time of diagnosis. An additional 10% of patients with a new diagnosis of EMPD are diagnosed with a noncontiguous malignancy within 12 months of diagnosis. The most commonly associated malignancies are cancers of the prostate, urinary tract, and breast. Associated carcinomas of the cervix, vagina, endometrium, bladder, and Bartholin glands have also been described. Due to these associations, a thorough investigation for underlying malignancy should accompany every diagnosis.

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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Diagnostic Pearls

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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.

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Therapy

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References

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Last Reviewed:09/11/2018
Last Updated:12/07/2021
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Extramammary Paget disease
See also in: Anogenital
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A medical illustration showing key findings of Extramammary Paget disease : Genitals, buttocks, perineum, Scaly plaque, Skin erosion, Inguinal region
Clinical image of Extramammary Paget disease - imageId=251815. Click to open in gallery. caption: 'An ill-defined pink plaque on the scrotum. Note the unrelated purple papules (angiokeratomas).'
An ill-defined pink plaque on the scrotum. Note the unrelated purple papules (angiokeratomas).

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