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      Cutaneous mucinosis in mixed connective tissue disease* Translated title: Mucinose cutânea na doença mista do tecido conjuntivo

      case-report

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          Abstract

          Cutaneous mucinosis is a group of conditions involving an accumulation of mucin or glycosaminoglycan in the skin and its annexes. It is described in some connective tissue diseases but never in association with mixed connective tissue disease. This report concerns two cases of cutaneous mucinosis in patients with mixed connective tissue disease in remission; one patient presented the papular form, and the other reticular erythematous mucinosis. These are the first cases of mucinosis described in mixed connective tissue disease. Both cases had skin lesions with no other clinical or laboratorial manifestations, with clinical response to azathioprine in one, and to an association of chloroquine and prednisone in the other.

          Translated abstract

          A mucinose cutânea é um grupo de condições em que há um acúmulo de mucina ou glicosaminoglicanos na pele e seus anexos. É descrita em algumas doenças do tecido conjuntivo, porem nunca em associação com doença mista do tecido conjuntivo. Relatamos dois casos de mucinose cutânea em pacientes com doença mista do tecido conjuntivo em remissão, um apresentava-se sob a forma papular e outro sob a forma reticular eritematosa de mucinose. Estes são os primeiros casos de mucinose descritos na doença mista do tecido conjuntivo. Ambos os casos apresentaram o quadro cutâneo de modo isolado, sem nenhuma outra manifestação clínico-laboratorial, havendo resposta à azatioprina em um e à cloroquina associada a prednisona no outro.

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          The new cutaneous mucinoses: a review with an up-to-date classification of cutaneous mucinoses.

          In the past years seven new cutaneous mucinoses have been described. These are briefly reviewed and included in a revised classification of cutaneous mucinoses.
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            Identification and molecular analysis of glycosaminoglycans in cutaneous lupus erythematosus and dermatomyositis.

            Glycosaminoglycans (GAGs), also known histologically as dermal mucin, accumulate in several inflammatory skin conditions. Because different GAG species have distinct immunologic effects, the authors examined two GAGs, hyaluronan (HA) and chondroitin sulfate (CS), using specific stains in cutaneous lupus erythematosus (CLE) and dermatomyositis (DM). In the dermis of one CLE subtype, tumid LE (TLE), they found only increased HA, but both HA and CS were significantly elevated in another CLE subtype, discoid LE (DLE). DM lesional dermis accumulated mainly CS but not HA. The authors then used glycomic gene expression microarrays to assess the expression of HA- and CS-related genes in CLE skin. Real-time quantitative PCR confirmed significantly increased expression of HAS2, CHSY1, and C4ST1 in the combined groups of CLE lesions (n = 8) compared to healthy controls (n = 4). Thus, the increase in HA in CLE presumably results from upregulation of HAS2, whereas CHSY1 and C4ST1 appear to contribute to increased CS. Based on their known immunomodulatory effects in other systems, HA and CS may thus participate in the pathophysiology of these inflammatory skin conditions.
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              Papulonodular dermal mucinosis in lupus erythematosus.

              We report two cases of lupus erythematosus (LE) in which a truncal papulonodular eruption predominated. Histologically the lesions were characterized by diffuse dermal mucin without the usual inflammatory or epidermal changes of LE. It is uncommon in LE for dermal mucin to be present in a sufficient quantity to produce a papulonodular eruption in the absence of typical epidermal changes.
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                Author and article information

                Journal
                An Bras Dermatol
                An Bras Dermatol
                An Bras Dermatol
                Anais Brasileiros de Dermatologia
                Sociedade Brasileira de Dermatologia
                0365-0596
                1806-4841
                Jul-Aug 2013
                : 88
                : 4
                : 635-638
                Affiliations
                [1 ] Rheumatologist, Staff Physician, Internal Medicine Department, University of São Paulo (USP) - São Paulo (SP), Brazil.
                [2 ] Rheumatologist; Rheumatology Department, University of São Paulo (USP) - São Paulo (SP), Brazil.
                [3 ] Pathologist, Dermatopathology Division, Department of Dermatology, University of São Paulo Teaching Hospital (USP) - São Paulo (SP), Brazil.
                [4 ] Staff Physician, Rheumatology Department, University of São Paulo (USP) - São Paulo (SP), Brazil.
                Author notes
                MAILING ADDRESS: Ana Paula Luppino Assad, Faculdade de Medicina da Universidade de São Paulo. Av. Dr. Arnaldo, 455, 3º andar, sala 3131, Cerqueira César 01246903 - São Paulo - SP Brazil E-mail: anastoamaro@ 123456yahoo.com.br
                Article
                10.1590/abd1806-4841.20131948
                3760946
                24068142
                e825cdca-c23e-4eb1-a8f2-39b1bee96659
                ©2013 by Anais Brasileiros de Dermatologia

                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
                : 17 June 2012
                : 19 September 2012
                Categories
                Case Report

                autoimmune diseases,lupus erythematosus, cutaneous,mixed connective tissue disease,mucinoses

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