Extramammary Paget Disease

Extramammary Paget Disease

Elsa F. Velazquez, MD

Large pale-staining neoplastic cells extensively infiltrate all levels of a hyperplastic epidermis in EMPD. There is a chronic inflammatory infiltrate in the upper dermis.

Cytokeratin 7 immunohistochemical stain strongly highlights the neoplastic cells throughout the epidermis. The use of CK7 may also assist in defining the margins of the disease.



  • Extramammary Paget disease (EMPD)


  • Adenocarcinoma involving epidermis and commonly extending to epithelium of eccrine glands &/or hair follicles (adenocarcinoma in situ)

  • May involve dermis in minority of cases



  • Uncertain in most cases

  • Cases limited to epidermis are postulated to originate from intraepidermal portion of sweat glands or from intraepidermal stem cells

  • Heterogeneous condition; can be classified as primary (cutaneous origin) and secondary (extracutaneous origin)

    • Rarely, primary EMPD is associated with underlying sweat gland adenocarcinoma (usually apocrine)

    • Secondary EMPD may represent extension from urethral, bladder, anal/rectal, prostatic, or endocervical carcinoma

      • Incidence of association with internal carcinoma is about 15%

      • Especially true in perianal EMPD, which is associated with rectal carcinoma in 1/3 of cases



  • Age

    • 6th-8th decade

  • Gender

    • Female predominance


  • Skin regions rich in apocrine glands

    • Most common sites are vulva and perianal region

    • Other sites include scrotum, perineum, penis, axilla, umbilicus

    • Exceptional sites include eyelid (accompanying carcinoma of Moll gland), external auditory meatus (associated with ceruminous carcinoma), face, scalp, chest, abdomen, and extremities


  • Circinate or annular moist erythematous scaly patches or plaques

  • Gray-white eczematous patches are also common

  • Hypopigmented macules, ulceration, crusting, or palpable tumor may be present

  • Pruritus is frequent

  • Bleeding, oozing, tenderness, or burning sensation can occur

  • EMPD may mimic eczema

  • Lesions may be multifocal


  • Complete surgical excision is treatment of choice for primary EMPD

  • Treatment in secondary lesions will depend on associated carcinoma


  • Primary EMPD confined to epidermis and epithelium of adnexal structures (in situ carcinoma) has good prognosis when completely excised

  • Patients require long-term follow-up because of multifocal nature of condition and high recurrence rate

  • Dermal invasion is associated with worse prognosis

  • Prognosis in secondary Paget disease is related to underlying carcinoma and is usually poor

Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Extramammary Paget Disease
Premium Wordpress Themes by UFO Themes
%d bloggers like this: