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==General== | ==General== | ||
*Two types | *Two types | ||
**Primary Extramammary Paget disease - | **Primary Extramammary Paget disease - thought to be an intraepidermal carcinoma arising from cutaneous apocrine glands | ||
***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. | ***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). | ***Usually entirely intraepidermal but may rarely be associated with an underlying apocrine gland carcinoma (in contrast to mammary Paget disease which is usually associated with underlying mammary carcinoma). | ||
**Secondary Extramammary Paget disease - intraepidermal spread from a distant tumour | **Secondary Extramammary Paget disease - intraepidermal spread from a distant tumour | ||
***Usually of urothelial or colorectal origin. | ***Usually of urothelial or colorectal origin. |
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