Difference between revisions of "Extramammary Paget disease"

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==General==
==General==
*Usually not associated with malignancy, unlike the ''Paget disease of the breast'' - '''important difference'''.
*Two types
*Classically seen in the [[vulva]].
**Primary Paget disease - a malignancy of the cutaneous apocrine glands
*May afflict [[penis]]<ref name=pmid19126202>{{Cite journal  | last1 = Ekwueme | first1 = KC. | last2 = Zakhour | first2 = HD. | last3 = Parr | first3 = NJ. | title = Extramammary Paget's disease of the penis: a case report and review of the literature. | journal = J Med Case Reports | volume = 3 | issue =  | pages = 4 | month =  | year = 2009 | doi = 10.1186/1752-1947-3-4 | PMID = 19126202 }}</ref> or [[scrotum]].<ref name=pmid24349803>{{Cite journal  | last1 = Guerra | first1 = R. | last2 = Misra | first2 = S. | title = Management of Extramammary Paget's Disease: A Case Report and Review of the Literature. | journal = Case Rep Dermatol Med | volume = 2013 | issue =  | pages = 436390 | month =  | year = 2013 | doi = 10.1155/2013/436390 | PMID = 24349803 }}</ref>
***Arises in apocrine rich areas - usually the vulva but also the groin, inguinal area, perineum, penis<ref name=pmid19126202>{{Cite journal  | last1 = Ekwueme | first1 = KC. | last2 = Zakhour | first2 = HD. | last3 = Parr | first3 = NJ. | title = Extramammary Paget's disease of the penis: a case report and review of the literature. | journal = J Med Case Reports | volume = 3 | issue =  | pages = 4 | month =  | year = 2009 | doi = 10.1186/1752-1947-3-4 | PMID = 19126202 }}</ref> or [[scrotum]].<ref name=pmid24349803>{{Cite journal  | last1 = Guerra | first1 = R. | last2 = Misra | first2 = S. | title = Management of Extramammary Paget's Disease: A Case Report and Review of the Literature. | journal = Case Rep Dermatol Med | volume = 2013 | issue =  | pages = 436390 | month =  | year = 2013 | doi = 10.1155/2013/436390 | PMID = 24349803 }}</ref> and rarely axilla or eye.
***Usually entirely intraepidermal but may be associated with an underlying apocrine gland carcinoma (in contrast to mammary Paget disease which is usually associated with underlying mammary carcinoma).
**Secondary Paget disease - intraepidermal spread from a distant tumor
***Usually of urothelial or colorectal origin.
***Arises in the perineal areas near these organs
 


Clinical:
Clinical:
*Pruritis.
*Pruritis.
*R/O VIN
*R/O vulvitis


==Gross==
==Gross==

Revision as of 10:39, 11 March 2015

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, scrotum, others

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

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

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

  • Two types
    • Primary Paget disease - a malignancy of the cutaneous apocrine glands
      • Arises in apocrine rich areas - usually the vulva but also the groin, inguinal area, perineum, penis[1] or scrotum.[2] and rarely axilla or eye.
      • Usually entirely intraepidermal but may be associated with an underlying apocrine gland carcinoma (in contrast to mammary Paget disease which is usually associated with underlying mammary carcinoma).
    • Secondary Paget disease - intraepidermal spread from a distant tumor
      • Usually of urothelial or colorectal origin.
      • Arises in the perineal areas near these organs


Clinical:

  • Pruritis.
  • R/O VIN
  • R/O vulvitis

Gross

Features:[2]

  • Plaque with an irregular border.
  • Erythematous or white.

Clinical DDx:

Images

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.[5]
  • 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. 2.0 2.1 2.2 Guerra, R.; Misra, S. (2013). "Management of Extramammary Paget's Disease: A Case Report and Review of the Literature.". Case Rep Dermatol Med 2013: 436390. doi:10.1155/2013/436390. PMID 24349803.
  3. Bansal, D.; Bowman, CA. (Feb 2004). "Extramammary Paget's disease masquerading as lichen sclerosus.". Int J STD AIDS 15 (2): 141-2. doi:10.1258/095646204322764361. PMID 15006079.
  4. URL: http://derm101.com/searchResults.aspx?searchStr=apocrine+carcinoma&rootTerm=apocrine+carcinoma&searchType=2&rootID=12687. Accessed on: 9 September 2011.
  5. 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.
  6. RS. May 2010.