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      Morbihan syndrome: a case report and literature review*

      case-report

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          Abstract

          Morbihan syndrome is a rare entity that more commonly affects women in the third or fourth decade of life. It is considered a special form of rosacea and its pathogenesis is not fully known. It is clinically characterized by the slow appearance of erythema and solid edemas on the upper portion of the face, with accentuation in the periorbital region, forehead, glabella, nose, and cheeks. We report the case of a patient presented with edema on the upper eyelid for a year. These findings suggested the diagnosis of Morbihan syndrome. We aim to report a rare, particularly refractory and chronic form of rosacea, which has received little attention in the literature.

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          Most cited references12

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          Rosacea - S1 guideline.

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            Morbihan disease.

            Morbihan disease, which consists of solid facial edema, is a rare complication of rosacea, a common cutaneous disorder in middle-aged individuals. The characteristic features of Morbihan disease are its chronic course, typical clinical picture, lack of specific laboratory and histopathologic findings, and refractoriness to therapeutic measures. Since its initial description in 1957, only a small number of cases have been reported in the dermatologic literature. We report a 54-year-old man who developed a two-year duration of erythema and edema that affects the upper and mid face, with accentuation in the periorbital region. Patch tests excluded an allergic contact dermatitis and histopathologic investigation showed small, nodular clusters of epithelioid cells in the dermis that were consistent with sarcoidal granulomata. A diagnosis of Morbihan disease was made owing to the combination of clinical and histopathologic findings. Therapeutic options for the disease remain unsatisfactory and treatments reported in the literature include systemic glucocorticoids, oral tetracyclines, thalidomide, isotretinoin, ketotifen, and clofazimine. Our patient failed a six-to-seven months course of minocycline prior to presentation and has since experienced improvement on gradually-increasing doses of isotretinoin.
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              Solid Persistent Facial Edema of Acne: Successful Treatment with Isotretinoin and Ketotifen

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                Author and article information

                Journal
                An Bras Dermatol
                An Bras Dermatol
                abd
                Anais Brasileiros de Dermatologia
                Sociedade Brasileira de Dermatologia
                0365-0596
                1806-4841
                Sep-Oct 2016
                Sep-Oct 2016
                : 91
                : 5 Suppl 1
                : 157-159
                Affiliations
                [1 ]Universidade de Santo Amaro (Unisa) – São Paulo (SP), Brazil
                [2 ]Faculdade de medicina de São José do Rio Preto (Famerp) – São Paulo (SP), Brazil
                Author notes
                Mailing address: Renata Trefiglio Eid, Rua Professor Enéas de Siqueira Neto, 320 Jardim das Imbuias, 04829-900 - São Paulo - SP Brazil. E-mail: renata_eid@ 123456hotmail.com
                Article
                10.1590/abd1806-4841.20164291
                5325027
                28300928
                8101cde3-9f42-4202-9f3d-2d84937fa1ed

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 27 January 2015
                : 15 April 2015
                Categories
                Case Report

                edema,erythema,rosacea
                edema, erythema, rosacea

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