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Extramammärer Morbus Paget

Lookup NU author(s): Dr Ioana Cosgarea

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Abstract

© 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.

Publication metadata

Author(s): Cosgarea I, Zaremba A, Hillen U

Publication type: Review

Publication status: Published

Journal: Der Hautarzt

Year: 2019

Volume: 70

Issue: 9

Pages: 670-676

Online publication date: 03/09/2019

Acceptance date: 02/04/2016

ISSN (print): 0017-8470

ISSN (electronic): 1432-1173

Publisher: Springer Verlag

URL: https://doi.org/10.1007/s00105-019-4459-8

DOI: 10.1007/s00105-019-4459-8

PubMed id: 31482274

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