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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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