Scleromyxedema: a case treated with oral prednisone

55 Case Report Scleromyxedema: a case treated with oral prednisone* Escleromixedema: um caso tratado com prednisona oral* Pedro Bezerra da Trindade Neto1 Aldavânea Cabral de Oliveira e Silva3 Alexandre de Oliveira Sales2 Juliana Cristina Soares Nunes4 Abstract: Scleromyxedema is an idiopathic cutaneous mucinosis characterized by a papular eruption, skin induration and paraproteinemia. Histologically, fibrolast proliferation can be observed in the upper dermis associated with a mucine deposition. Treatment is difficult and at present there is no totally effective therapeutic modality to control the disease. The present report is on a 68-year-old patient with scleromyxedema without systemic manifestation, who responded to oral steroid therapy. Keywords: Adrenal cortex hormones; Hyaluronic acid; Hypergammaglobulinemia; Mucinoses Resumo: O escleromixedema é uma mucinose cutânea idiopática caracterizada por erupção papulosa, induração da pele e paraproteinemia. Histologicamente, se observa proliferação de fibroblastos na derme superior associada a depósito de mucina. O tratamento é difícil, não existindo na atualidade modalidade terapêutica totalmente eficaz para controlar a enfermidade. Relata-se o caso de um paciente de 68 anos com escleromixedema, sem manifestação sistêmica, que respondeu à terapia oral com corticosteróide. Palavras-chave: Ácido hialurônico; Corticosteróides; Hipergamaglobulinemia; Mucinoses INTRODUCTION Scleromyxedema is a primary cutaneous mucinosis of unknown origin, characterized by infiltrating lesions in the skin, a mucine deposit in the upper dermisis and monoclonal paraproteinemia.1,2 The myxedematous lichen spectre (papular mucinosis) was originally described by Dubreuilh and Reitman, in 1906.3 In 1953, Montgomery and Underwood4 classified the myxedematous lichen into four clinical forms: (1) generalized lichenous papular eruption; (2) nonconfluent papular form; (3) localized or generalized lichenous plaques; and (4) urticaria-like plaques and nodular lichenous eruptions. In 1954, Gottron and Arndt introduced the term scleromyxedema to refer to a variant of papular mucinosis which corresponds to a generalized diffuse lichenous eruption with skin sclerosis and normal thyroid function.1,5 As a response to the indiscriminate use of the terms papular mucinosis, myxedematous lichen, and scleromyxedema, in 2001, Rongioletti et al.,6 based on anatomic-clinical criteria, adopted a new classification for this group of diseases: (1) generalized myxedematous lichen (scleromyxedema); (2) localized myxedematous lichen; (3) atypical forms of myxedematous lichen. Even though there have been reports of diverse therapeutic regimens, the benefits for this condition are variable.7 The objective of this paper is to describe a case of this rare entity, stressing the favorable clinical response to the systemic use of a corticosteroid. CASE REPORT 68-year-old male, anos, do sexo masculino, married, agricultural worker, born in and coming from Received on August 01, 2004. Approved by the Consultive Council and accepted for publication on December 07, 2005. * Work done at Dermatology Service of the Hospital Universitário Onofre Lopes/Universidade Federal do Rio Grande do Norte – UFRN - (RN), Brazil Conflict of interests: None 1 2 3 4 Associate Professor of Dermatology at the Universidade Federal do Rio Grande do Norte – UFRN – Rio Grande do Norte (RN), Brazil. Ph.D. in Dermatology from the Universidade Federal do Rio de Janeiro – UFRJ – Rio de Janeiro (RJ), Brazil. Pathologist at the Hospital Universitário Onofre Lopes Universidade Federal do Rio Grande do Norte – UFRN – Rio Grande do Norte (RN), Brazil. Resident Physician at Dermatology Service of the Hospital Universitário Onofre Lopes - Universidade Federal do Rio Grande do Norte – UFRN – Rio Grande do Norte (RN), Brazil. Resident Physician at Dermatology Service of the Hospital Universitário Onofre Lopes - Universidade Federal do Rio Grande do Norte – UFRN – Rio Grande do Norte (RN), Brazil. ©2006 by Anais Brasileiros de Dermatologia An Bras Dermatol. 2006;81(1):55-8. 56 Trindade Neto PB, Sales AO, Silva ACO, Nunes JCS. Espirito Santo, state of Rio Grande do Norte. For one year the patient has presented confluent erythema accompanied by slight pruritus, inicially on the face and upper portion of the thorax, extending to the rest of the body, with the further appearance of progressive skin hardening. The dermatological exam revealed diffuse skin infiltration associated to multiple papules, sparse or confluent, with diameters ranging from 2 to 4 mm, located symmetrically on the extending surface of the arms, retroauricular areas (Figure 1) thorax and face, with intensification in the forehead folds and glabellar area (Figure 2). A scarcity of hair was also observed in the eyebrows and axillae. Hemogram and liver, kidney and thyreoid functions were normal. Electrophoresis of serum proteins revealed hypergammaglobulinemia. Quantitatively, there was an increase of IgG immunoglobulin: 2.110mg/dl (normal: 564-1765mg/dl), but with normal values for IgE, IgM, and IgA. Serum immunoelectrophoresis by immunofixation showed monoclonal paraprotein of the IgG type with a predominance of the lambda chain. The histopathological examination of the skin showed a separation of collagen beams by a slightly basophilous amorphous material in the high papillar and reticular dermis, with a proliferation of enlarged fusiform fibroblasts (Figure 3). By means of alcian blue and colloidal iron dyeing the mucine deposit was made evident. (Figure 4). The diagnostic criteria proposed for scleromyxedema included the presence of skin infiltration, the deposition of mucinous material on the dermis, exclusion of clinical and lab-tested thyroid dysfunction, as well as demonstration of paraproteinemia. FIGURE 1: Small lichenous papules behind the ear An Bras Dermatol. 2006;81(1):55-8. The patient began treatment with prednisone, 60mg/day, having had previous prophylaxis for disseminated strongiloydiasis with cambendazol (5 mg/kg) at a single dose. The second month of follow-up showed a reduction of erythema and pruritus. After six months there was a regression of papular eruption, skin infiltration, and IgG paraprotein was reduced to 1.11 ng/dl. By the ninth month of prednisone therapy and the clinical improvement of the lesions (Figure 5), steroid reduction was begun. DISCUSSION Scleromyxedema is an idiopathic cutaneous mucinosis with a chronic, progressive course, characterized by general papular eruption with skin induration, frequently associated with a peculiar monoclonal paraproteinemia (83.2% of cases).6 The role of paraproteinemia in the pathogenesis of this disease remains unclarified. Habitually, it is a lambda chain IgG, although some patients may present kappa chain or IgA.5 The paraproteinemia differs from normal IgG because it is extremely basic globulin of a small size (molecular mass: 110Kda; normal: 160Kda)8 and becaus (...truncated)