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code of breast: XH3E21: extramammary: XH70F8
3.3.3 Paget’s Disease
Grading & Level of Importance: B
ICD-11
Epidemiology
Rare disease. 3% of all breast cancers.
Definition
Uncommon intraepidermal adenocarcinoma, arising from intraductal carcinoma in the breast (mammary Paget’s disease) Can also arise from apocrine sweat glands or underlying carcinomas e.g. rectum, prostate (extramammary Paget’s disease).
Aetiology & Pathogenesis
Associated breast cancer is of the intraduct variety.
Signs & Symptoms
Circumscribed, erythematous, sometimes scaly, increasingly infiltrated plaque. Nipple (unilateral), axilla, groin or perianal. Caution: for chronic dermatitis involving one nipple -> rule out Paget’s disease.
Localisation
Nipple, axilla, genitalia, lower abdomen, perianal.
Dermatopathology
Large clear intraepithelial tumour cells, PAS positive.
Course
Slowly and gradually spreads.
Complications
Association with intraductal carcinoma (Paget’s disease) or adenocarcinoma (extramammary Paget’s disease).
Diagnosis
Clinical features and histology.
Differential diagnosis
Nipple dermatitis, perianal dermatitis, psoriasis.
Prevention & Therapy
Surgical excision adjusted to tumour size and underlying disease, multidisciplinary approach.
Differential Diagnosis
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