Extramammary Paget disease

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Extramammary Paget disease, abbreviated EMPD, is a skin disease. As the name suggests, there is also a Paget disease of the breast.

Extramammary Paget disease
Diagnosis in short

Extramammary Paget's disease. H&E stain.

LM large epithelioid cells - nested or single - in the epidermis, clear/pale cytoplasm (occasionally eosinophilic), large nucleoli
LM DDx benign Toker cell hyperplasia, malignant melanoma, Bowen's disease, apocrine carcinoma of the skin
IHC CK7 +ve, CEA +ve, S-100 -ve, CK5/6 -ve, HER2 +ve
Gross erythema, +/-weeping, +/-crusted
Site vulva, penis

Symptoms pruritis (itchy)
Prognosis typically benign - usually not associated with an underlying malignancy (unlike Paget's disease of the breast)
Clin. DDx contact dermatitis, lichen sclerosus

There is also a Paget disease of the bone - just to make things confusing. This is dealt with in the bone article and has nothing (from a pathologic perspective) to do with the Paget disease discussed in this article

General

  • Usually not associated with malignancy, unlike the Paget disease of the breast - important difference.
  • Classically seen in the vulva.
  • May afflict penis.[1]

Clinical:

  • Pruritis.

Gross

  • Erythema.

Microscopic

Features:

  • Epitheliod morphology (round/ovoid).
  • Cells nested or single.
  • Clear/pale cytoplasm key feature - may also be eosinophilic.
  • Large nucleoli.

Images

DDx

Stains

IHC

Panel:

  • CEA +ve (-ve in Bowen's disease, -ve in Toker cells).
  • CK7 +ve.
    • Toker cells CK7 +ve.[3]
  • S100 -ve, HMB-45 -ve (both typically +ve in melanoma).

Additional:

See also

References

  1. Ekwueme, KC.; Zakhour, HD.; Parr, NJ. (2009). "Extramammary Paget's disease of the penis: a case report and review of the literature.". J Med Case Reports 3: 4. doi:10.1186/1752-1947-3-4. PMID 19126202.
  2. URL: http://derm101.com/searchResults.aspx?searchStr=apocrine+carcinoma&rootTerm=apocrine+carcinoma&searchType=2&rootID=12687. Accessed on: 9 September 2011.
  3. Nofech-Mozes, S.; Hanna, W.. "Toker cells revisited.". Breast J 15 (4): 394-8. doi:10.1111/j.1524-4741.2009.00743.x. PMID 19601945.
  4. RS. May 2010.