By Gary M. White, MD
Courtesy Ann McGowan-Tuskes, MD
Extramammary Paget's disease (EMPD) is a neoplasm of the skin that histologically mimics Paget's disease of the breast. EMPD may be categorized as follows:
Extramammary Paget's disease (EMPD) presents as an exudative eczematous or red plaque in the groin, perianal area, or axilla. In one study, the frequency of occurrence in these different sites was genital 91%, axillary 6%, perianal 5%, and ectopic 1%. (The reason it adds up to more than 100 is that some patients had multiple sites involved.) EMPD has also been reported in the oral cavity, ear (associated with ceruminous gland carcinoma), eyelid margin (associated with carcinoma of Moll's glands), cheek, buttocks, back, chest, and umbilicus. These cases outside the typical areas of the genital, axillary, and perianal region have been termed ectopic EMPD. One case of ectopic EMPD was pigmented (BJD 1996;134;958). It most commonly occurs from the sixth to the eighth decade.
Verrucous areas may develop within the plaque. Rarely, depigmented macules may accompany or be the sole manifestation of EMPD [BJD 1994;130;101(CP)].
EMPD may occur in multiple locations when a neoplasm spreads or metastasizes to multiple sites. It may also very rarely occur as multifocal malignant transformation [Dermatology Online Journal 2016;22(1)].
Refer to urology and gastroenterology for cancer screening. Patients should have a colonoscopy. Women should have a full examination by a gynecologist and have a mammogram.
Surgical excision with care to obtain adequate margins is recommended. The highest cure rate seems to be Mohs microsurgery enhanced with CK7 staining of frozen sections. In one series of 49 cases [Dermatology News June 2016 p. 35, Dr Damavandy University of Pennsylvania, Philadelphia] there was a 5 year recurrence-free cure rate of 95%. Without Mohs surgery, 5-7 cm of normal skin is needed to have a relatively high cure rate [JAAD 2004;51:767-73]. In most cases, this large a margin is prohibitive. Postoperative radiation therapy is safe and effective in maintaining local control [BJD 2015;172;1014].
Radiotherapy is a reasonable alternative for elderly patients with inoperable lesions (Ann Oncol. 2014 Jan;25(1):291-7).
A study of 21 women with vulvar EMPD treated with imiquimod concluded that when associated cancers and invasive growth are excluded, imiquimod appears to be a useful treatment option for recurrent EMPD (JAAD April 2014)
Patients with invasive EMPD are at higher risk of secondary malignancies--mostly due to a significantly increased incidence of colorectal and anal malignancies (BJD March 2013). Long-term followup therefore is in order.
Man in his 50's with a slowly growing red, itchy area (left scrotum) over 2 years.
65-year-old woman with a moist plaque in the right axilla. J Cut Aesth Surgery 2014;7:131-134
47-year-old woman with a slowly growing ulcerated plaque in the left groin. Virtual Grand Rounds in Dermatology April 30, 2008
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