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      Case for diagnosis. Lichen myxedematosus*

      case-report

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          Abstract

          Scleromyxedema or lichen myxedematosus is a rare papular mucinosis of chronic and progressive course and unknown etiology. It is commonly associated with monoclonal gammopathy and may show extracutaneous manifestations, affecting the heart, lung, kidney, and nerves. The diagnosis is based on four criteria: generalized papular and sclerodermoid lesions; mucin deposition, fibroblast proliferation, and fibrosis in the histopathology; monoclonal gammopathy; and no thyroid disorders. This article reports the case of a scleromyxedema patient with a recent history of acute myocardial infarction and monoclonal gammopathy.

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          Most cited references16

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          [Papular mucinosis associated with hypothyroidism].

          Morgana Volpato, Tatiana Jaime, Martha Pisani Proença … (2010)
          Papular mucinosis or lichen myxedematosus is an idiopathic disorder characterized by papules, nodules or plaques caused by mucin deposition in the dermis, in the absence of thyroid disease. The present report describes an atypical case with exuberant lesions in the form of plaques, associated with subclinical hypothyroidism. In cutaneous mucinosis, there is an abnormal quantity of mucin in the dermis. In traditional classifications of papular mucinosis, an absence of thyroid disease constitutes a diagnostic criterion. A few cases of the disease have been reported in association with hypothyroidism constituting, as in the present case, atypical lichen myxedematosus. It is therefore suggested that the presence of thyroid disease should not be considered an exclusion criterion for the diagnosis of this condition.
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            Dermatology

            M Ramos-E-Silva, MC RIBEIRO, J Bolognia … (2012)
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              Lichen myxoedematosus with associated cardiac abnormalities.

              Jinuraj Rajappan, Sean D. Sheridan, Timothy Hardman … (2001)
              We describe a 42-year-old woman who developed lichen myxoedematosus. Twenty years after the onset of the disease she became breathless and hypertensive, and an echocardiogram showed a mass on the mitral valve, which was thought to be a mucin deposit. Her hypertension was resistant to treatment with combination antihypertensives. To our knowledge, this is the first report to link lichen myxoedematosus with a valvular mucinous mass. This case also demonstrates the slow clinical progression of the disease over 20 years.
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                Author and article information

                Journal
                Journal ID (nlm-ta): An Bras Dermatol
                Journal ID (iso-abbrev): An Bras Dermatol
                Journal ID (publisher-id): abd
                Title: Anais Brasileiros de Dermatologia
                Publisher: Sociedade Brasileira de Dermatologia
                ISSN (Print): 0365-0596
                ISSN (Electronic): 1806-4841
                Publication date (Print and electronic): Nov-Dec 2016
                Publication date (Print): Nov-Dec 2016
                Volume: 91
                Issue: 6
                Pages: 842-843
                Affiliations
                [1 ] Faculdade Evangélica do Paraná (Fepar) – Curitiba (PR), Brazil
                [2 ] Universidade Positivo (UP) – Curitiba (PR), Brazil
                [3 ] Universidade Federal do Paraná (UFPR) – Curitiba (PR), Brazil
                Author notes
                Mailing address: Priscila Regina Orso Rebellato, Rua Luiz Leitner, 50, Bairro Bigorrilho, 80710-390 - Curitiba - PR Brazil, E-mail: prirebellato@ 123456yahoo.com.br
                Article
                DOI: 10.1590/abd1806-4841.20165725
                PMC ID: 5193206
                PubMed ID: 28099617
                SO-VID: 768e3fe9-b2e8-42a1-a9fc-af17b8a6397c
                License:

                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
                Date received : 23 February 2016
                Date accepted : 12 June 2016
                Categories
                Subject: What Is Your Diagnosis?

                Keywords: mucinoses,paraproteinemia, scleromyxedema
                Data availability:
                Keywords: mucinoses, paraproteinemia, scleromyxedema

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