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Lookup NU author(s): Dr Ioana Cosgarea
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© 2019, Springer Medizin Verlag GmbH, ein Teil von Springer Nature.Extramammary Paget’s disease (EPD) is a rare, slowly growing, cutaneous adenocarcinoma with an incidence of 0.1–2.4 per 1,000,000 inhabitants. Histologically, EPD is characterized by the presence of epidermal Paget’s cells, similarly to mammary Paget’s disease. The EPD is typically divided into primary EPD (type I) and secondary EPD (type II associated with colorectal carcinoma and type III associated with urogenital carcinoma). From a clinical point of view, EPD is unspecific commonly mimicking chronic inflammatory skin disorders. This unspecific clinical picture can impede and delay the diagnosis of EPD. The treatment of choice for local EPD is the micrographically controlled excision. The extent of the infiltration of adnexal structures should be histologically determined prior to topical therapies, such as imiquimod and superficial ablative therapy. The complete excision of the tumor can be challenging due to ill-defined borders. In the metastatic stage the EPD has a poor prognosis. Controlled clinical trials for systemic treatment are still lacking.
Author(s): Cosgarea I, Zaremba A, Hillen U
Publication type: Review
Publication status: Published
Journal: Der Hautarzt
Online publication date: 03/09/2019
Acceptance date: 02/04/2016
ISSN (print): 0017-8470
ISSN (electronic): 1432-1173
Publisher: Springer Verlag
PubMed id: 31482274