Extramammary Paget disease
Extramammary Paget's Disease (EMPD)
Definition & Pathology
- EMPD = apocrine-differentiated intraepithelial adenocarcinoma with slow pagetoid spread in the epidermis.
- Patients: Women > Men, ~ 70 yrs. — usually in the 7th decade of life.
- Location: apocrine areas (anogenital + axillary) — also perineal, inguinal, periumbilical.
Clinical Presentation
- Solitary, sharply demarcated erosive-weeping plaque with a finely scaling surface, possibly mild pruritus.
- Common misdiagnoses: Eczema + Inverse Psoriasis — months of delay.
Histology & IHC
- "Pagetoid clear cells" intraepithelial (also deeper in adnexal structures).
- IHC: CK7+ + CEA+ + GCDFP-15+ — Differentiation from SCC (CK7−) and melanoma (S100+, CK7−).
- Subclassification: primary cutaneous (intraepithelial) vs. secondary as spread of an internal adenocarcinoma (rectum, urothelium, breast) into the skin.
Diagnostics / Staging
- Punch biopsy + mapping biopsies for extent determination (often much larger than clinically visible!).
- Exclude secondary adenocarcinoma:
- Anogenital / Perianal: Rectoscopy + colonoscopy (in case of perianal involvement)
- Penis / Scrotum: Urogenital diagnostics (cystoscopy, ultrasound)
- Inguinal / Vulva: Gynecology + Urology consultation
- Mammography for axillary localization
- CT / Ultrasound of lymph node stations for metastasis search.
Differential diagnoses
- Bowen's Disease
- Mammary Paget disease
- Psoriasis vulgaris (plaque psoriasis)
- Basal cell carcinoma
- Seborrhoeic dermatitis
- Melanoma
- Atopic dermatitis (atopic eczema)
- Allergic contact dermatitis
Practise Extramammary Paget disease in the app
Flashcards with spaced repetition, exam questions and spot-the-diagnosis on this topic – in DermaFuchs, free of charge.
In the DermaFuchs app: 4 flashcards · 4 clinical images
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Note: This page is intended for medical education and does not replace diagnosis or treatment decisions in individual cases. Treatment and follow-up content is available in the app.