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==
653

edits

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