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| {{CMG}}{{AE}}{{Simrat}} | | {{CMG}}{{AE}}{{Simrat}} |
| ==Overview== | | ==Overview== |
| The cause of extramammary Paget's disease has not been identified.<ref>Lever, Walter F., and David E. Elder. Lever's histopathology of the skin. Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins, 2009.</ref><ref name="pmid11064666">{{cite journal| author=Lloyd J, Flanagan AM| title=Mammary and extramammary Paget's disease. | journal=J Clin Pathol | year= 2000 | volume= 53 | issue= 10 | pages= 742-9 | pmid=11064666 | doi= | pmc=PMC1731095 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11064666 }} </ref><ref>Mehregan, Amir H., and Hermann Pinkus. Pinkus' guide to dermatohistopathology. Norwalk, Conn: Appleton-Century-Crofts, 1986. Print.</ref><ref name="OrlandiPiccione2001">{{cite journal|last1=Orlandi|first1=A.|last2=Piccione|first2=E.|last3=Francesconi|first3=A.|last4=Spagnoli|first4=L. G.|title=Simultaneous vulvar intraepithelial neoplasia and Paget's disease: Report of two cases|journal=International Journal of Gynecological Cancer|volume=11|issue=3|year=2001|pages=224–228|issn=1048-891X|doi=10.1046/j.1525-1438.2001.01016.x}}</ref> | | The direct cause of extramammary Paget's disease has not been identified. |
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| ==Causes== | | ==Causes== |
| *The cause of Extramammary Paget's disease (EMPD) is unknown. | | *The direct cause of Extramammary Paget's disease (EMPD) is unknown. |
| *The most popular theory is that extramammary Paget's disease (EMPD) may arise either as a primary intraepidermal neoplasm of the epidermis (i.e, primary EMPD) or less commonly as a result of spread from an underlying internal malignancy (i.e, secondary EMPD).
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| *In primary EMPD, Paget's cells probably originate from primitive basal cells within the [[epidermis]] or from intraepidermal portions of [[apocrine glands]]. Primary Paget's disease may progress from in situ intraepidermal neoplasia to dermally invasive [[adenocarcinoma]], which may in turn metastasise to distant sites and local [[lymph nodes]].
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| *In secondary EMPD, Paget's disease arise following epidermotropic spread of malignant cells from an underlying neoplasm in a dermal adnexal gland or a local internal organ with contiguous epithelium.
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| *Some investigators believe that EMPD may be associated with a generalised tendency to neoplasia, especially [[adenocarcinoma]], as there is a high rate of metasynchronous and synchronous cancers in these patients. An association of EMPD of the vulva and high grade vulval intraepithelial neoplasia (VIN) has been reported by some authors.<ref>Lever, Walter F., and David E. Elder. Lever's histopathology of the skin. Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins, 2009.</ref><ref name="pmid11064666">{{cite journal| author=Lloyd J, Flanagan AM| title=Mammary and extramammary Paget's disease. | journal=J Clin Pathol | year= 2000 | volume= 53 | issue= 10 | pages= 742-9 | pmid=11064666 | doi= | pmc=PMC1731095 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11064666 }} </ref><ref>Mehregan, Amir H., and Hermann Pinkus. Pinkus' guide to dermatohistopathology. Norwalk, Conn: Appleton-Century-Crofts, 1986. Print.</ref><ref name="OrlandiPiccione2001">{{cite journal|last1=Orlandi|first1=A.|last2=Piccione|first2=E.|last3=Francesconi|first3=A.|last4=Spagnoli|first4=L. G.|title=Simultaneous vulvar intraepithelial neoplasia and Paget's disease: Report of two cases|journal=International Journal of Gynecological Cancer|volume=11|issue=3|year=2001|pages=224–228|issn=1048-891X|doi=10.1046/j.1525-1438.2001.01016.x}}</ref>
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| ==References== | | ==References== |
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