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Tumour · Adnexal · ApocrineICD-10 C44

Extramammary Paget's disease

EMPD; extramammary Paget disease

Extramammary Paget's disease is a rare intraepithelial adenocarcinoma of apocrine-bearing skin — most commonly vulva, perianal, scrotum and axilla. Two forms exist: primary (originating from intraepidermal apocrine cells) and secondary (representing pagetoid spread from an underlying GI, GU or other adenocarcinoma). Differentiating these has critical management implications.

CurrentLast reviewed 25 March 2026
Clinical image of Extramammary Paget's disease
Extramammary Paget's disease. Image sourced from DermNet New Zealand. Used under CC BY-NC-ND 4.0. No endorsement implied.

Clinical features

  • Slowly enlarging well-demarcated erythematous plaque, often eczematous, scaly or weeping.
  • Frequently misdiagnosed as eczema, intertrigo, candida or psoriasis — diagnostic delay is the rule.
  • Pruritus is the predominant symptom; may be tender or burning.
  • Most common sites: vulva (most common; ~65% of all EMPD cases), perianal, scrotum / penile shaft, axilla.

Diagnosis

Punch biopsy is essential. Histology shows pagetoid intraepidermal adenocarcinoma cells with abundant pale cytoplasm. Immunohistochemistry distinguishes primary from secondary disease and excludes pagetoid melanoma:

  • Primary EMPD: CK7+, CK20−, GCDFP-15+ (apocrine origin).
  • Secondary EMPD (anorectal): CK7+, CK20+, CDX2+ → pagetoid spread from colorectal adenocarcinoma.
  • Secondary EMPD (urothelial): CK7+, CK20+, uroplakin III+, GATA3+.
  • Pagetoid melanoma: S100+, SOX10+, CK7−.

Work-up for underlying malignancy

Approximately 25% of EMPD cases (especially perianal) are secondary to an underlying internal malignancy. Investigations should be tailored to site:

  • Vulval / penile EMPD: pelvic examination, cervical screening up to date.
  • Perianal EMPD: colonoscopy mandatory; consider transvaginal / transrectal ultrasound; PSA in men.
  • Axillary EMPD: mammography, breast examination.
  • Scrotal / penile: urological work-up (urinalysis, cystoscopy if symptomatic).

Management

Surgery

  • Wide local excision with histologically clear margins is gold standard for primary EMPD — but subclinical extension is the rule and re-excision often required.
  • Mohs micrographic surgery with CK7 immunostaining offers superior margin assessment and is increasingly used.
  • Reconstruction often requires plastic surgery input — vulval split-thickness skin graft, perineal flap, etc.

Non-surgical (selected)

  • Topical imiquimod 5% — moderate response rates (~50%); option for elderly / unfit / declining surgery.
  • Photodynamic therapy.
  • Radiotherapy — for inoperable disease or palliation.

Recurrence and follow-up

Local recurrence rate is high (30–50% after wide excision; ~10–20% after Mohs). Long-term follow-up at 6–12 monthly intervals is essential.

Prognosis

Primary EMPD without invasion: excellent prognosis but high local recurrence. Invasive primary EMPD (lamina propria invasion, dermal invasion): worse prognosis, nodal metastatic potential. Secondary EMPD prognosis dictated by the underlying carcinoma.

References

  1. Lopes Filho LL et al. Mammary and extramammary Paget's disease. An Bras Dermatol; 2015.
  2. Edey KA et al. Interventions for the treatment of Paget's disease of the vulva. Cochrane Database Syst Rev; 2013.

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