By Disease Name > Extramammary Paget's Disease

Extramammary Paget's Disease

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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;  Bowens 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 Molls 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: Bowens disease, superficial spreading melanoma:

Pagets cells: CEA+ (a sweat gland marker), mucin stains + ( i.e. Hales colloidal iron, PAS, alcian blue at 2.5); no dyskeratosis
Bowens: anti-keratin stains; dyskeratosis
melanoma: anti-S100 protein stains