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      A Case Report of Familial Extramammary Paget's Disease in Female Siblings

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          Abstract

          Extramammary Paget's disease (EMPD) is a rare intraepithelial neoplasm that occurs in apocrine-bearing areas of skin. Most EMPD patients initially present with chronic pruritic eczematous lesions involving genitalia, perineum and perianal area. Familial form of EMPD is extremely rare. Several genetic mutations have been proposed but specific modes of inheritance are still unknown. This article reports two cases of familial extramammary Paget's disease in female siblings.

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          Most cited references19

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          Extramammary Paget disease.

          In 1874, Sir James Paget first described Paget disease of the nipple, also known as mammary Paget disease. In 1889, extramammary Paget disease (EMPD) of the scrotum and penis was identified. Although mammary and extramammary Paget disease are both characterized by epidermal Paget cells and share a similar clinical presentation, their uniqueness lies in anatomical location and histogenesis. EMPD presents as an erythematous plaque on apocrine gland bearing areas (i.e. vulva, perineum, perianal region, scrotum, and penis) in older men and women. It can be a focal, multifocal, or an ectopic process. Immunohistochemical staining allows for differentiation between primary and secondary EMPD in addition to the many other disease entities that clinically resemble this malignancy. When diagnosing a patient with EMPD, a full history and physical should be performed given the possibility of an underlying malignancy. Surgical excision currently is first line therapy and the prognosis is often favorable. Recent advances within the field have examined the expression of chemokine receptors within tumors, which may be applicable in determining prognosis. This review addresses the history, epidemiology, pathogenesis, clinical presentation, histopathology, differential diagnosis, diagnosis, management, and new observations with respect to extramammary Paget disease.
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            Oncogenic mutations in extramammary Paget's disease and their clinical relevance

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              The role of immunohistochemistry in discriminating primary from secondary extramammary Paget disease.

              Extramammary Paget disease (EMPD) is categorized into 2 groups: primary EMPD or EMPD secondary to underlying malignancy. Primary EMPD has a better prognosis, and the ability to distinguish between the 2 subsets has clinical relevance. Recent studies have suggested that immunostains, including cytokeratin (CK) 7, CK20, and BRST-2, distinguish between the 2 groups. We analyzed a large series of EMPD with an expanded immunohistochemical panel to assess its value in distinguishing primary from secondary disease.
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                Author and article information

                Journal
                Case Rep Dermatol
                Case Rep Dermatol
                CDE
                Case Reports in Dermatology
                S. Karger AG (Allschwilerstrasse 10, P.O. Box · Postfach · Case postale, CH–4009, Basel, Switzerland · Schweiz · Suisse, Phone: +41 61 306 11 11, Fax: +41 61 306 12 34, karger@karger.com )
                1662-6567
                Jan-Apr 2021
                22 March 2021
                22 March 2021
                : 13
                : 1
                : 176-183
                Affiliations
                Department of Dermatology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand
                Author notes
                *Charussri Leeyaphan, Department of Dermatology, Faculty of Medicine Siriraj Hospital, Mahidol University, 2 Wanglang Road, Bangkok Noi, Bangkok 10700 (Thailand), charussrilee@ 123456gmail.com
                Article
                cde-0013-0176
                10.1159/000514253
                8488423
                34703424
                5c618b67-edba-43af-9f8a-fde71ce0d8a9
                Copyright © 2021 by S. Karger AG, Basel

                This article is licensed under the Creative Commons Attribution-NonCommercial-4.0 International License (CC BY-NC) (http://www.karger.com/Services/OpenAccessLicense). Usage and distribution for commercial purposes requires written permission.

                History
                : 4 November 2020
                : 27 December 2020
                : 2021
                Page count
                Figures: 4, Tables: 1, References: 19, Pages: 8
                Categories
                Case Series

                Dermatology
                familial extramammary paget's disease,extramammary paget's disease,paget's disease of the vulvar

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