• | predilection for areas with high density of apocrine glands: vulva, scrotum, perianal, and axillae |
• | anogenital involvement most common |
• | any “eczematous” area in apocrine gland-bearing skin that does not resolve with appropriate therapy must make you think about Paget’ s disease |
• | clinical: looks acute eczematous or erosive; often pruritic |
• | ddx: LSC; Bowen’s disease; candida or other intertrigo |
relationship between EMPD and malignancy:
• | in more than 50% of cases no underlying malignancy is found |
• | but must work up for adnexal or visceral carcinoma |
adnexal carcinoma:
• | include apocrine gland carcinoma, eccrine gland carcinomas, sebaceous carcinoma of the eyelid, cancer of Moll’s gland of the eyelid , and carcinoma of the ceruminous gland |
visceral carcinoma:
• | genitourinary or gastrointestinal tract |
• | seems to be a relationship between the site of EMPD and the anatomic site of the visceral carcinoma (e.g. perianal EMPD, think rectal CA; EMPD of penis, think of genitourinary tract CA) |
histologic ddx: Bowen’s disease, superficial spreading melanoma:
• | Paget’s cells: CEA+ (a sweat gland marker), mucin stains + ( i.e. Hales colloidal iron, PAS, alcian blue at 2.5); no dyskeratosis |
• | Bowen’s: anti-keratin stains; dyskeratosis |
• | melanoma: anti-S100 protein stains |
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