Scleromyxedema (Arndt-Gottron Syndrome): a Case Report

Abstract

associated with systemic, even lethal manifestations, fi broblast proliferation and accumulation of acid mucopolysaccharides of the hyaluronic acid. Serum IgG class paraproteinemia is always present and it can be detected in all patients after appropriate or repeat testing (1,2,3).A case of an adult male with IgG paraproteinemia, in whom lichenoid papules evolved to scleromyxedema after two years, is reported. L ichen myxedematosus, also known as papular mucinosis, is a primary diff use cutaneous mucinosis. It is a rare cutaneous myxedematous condition characterized by formation of numerous lichenoid papules. Scleromyxedema, or ArndtGottron syndrome, is a rare, confl uent, papular and sclerotic variant of lichen myxedematosus, which is characterized by diff use thickening of the skin that underlies the papules. Th e condition is AbstractLichen myxedematosus, also known as papular mucinosis, is a primary diffuse cutaneous mucinosis. It is a rare cutaneous myxedematous condition characterized by formation of numerous lichenoid papules. Scleromyxedema, also known as Arndt-Gottron syndrome, is a rare, confl uent, papular and sclerotic variant of lichen myxedematosus, characterized by diffuse thickening of the skin underlying the papules. The condition is associated with systemic, even lethal manifestations, fi broblast proliferation and accumulation of acid mucopolysaccharides of the hyaluronic acid. Serum IgG class paraproteinemia is always present and it can be detected in all patients if appropriate or even repeat testing is used. Herein, we present a 67-year-old patient with a 2-year history of skin problems. He had no health problems other than hypertension and diabetes, wich were both diagnosed 15 years before. On examination, the patient exhibited sclerodermoid lesions with diffuse pseudo-sclerodermatous thickening of the exposed skin, microstomia and sclerodactyly-like changes; on the face, there were numerous solid, shiny 2 -4 mm in diameter skin-coloured lichenoid papules, scattered across the forehead, glabellar area, nasolabial folds, perioral region, ear lobes and the neck. Histopathological examination revealed: highly distinctive collagenosis and fi brosis in the middle dermis, increased fi broblasts; collagen bundles with irregular arrangement and fragmentation; alcian blue-positive deposits with appearance consistent with acid mucins. Serum protein electrophoresis detected IgG lambda paraproteinemia. The patient was treated with systematic corticosteroids during 9 months with subsequent introduction of methotrexate and showed satisfactory results. The etiology of scleromyxedema remains unknown, since the purifi ed IgG paraprotein itself has no direct effects on fi broblast proliferation. In scleromyxedema, numerous therapeutic modalities are proposed, unfortunatelly with limited effects. In colclusion, we report a case of an adult male with lichenoid papules; after a two-year progression, they evolved into scleromyxedema and exhibited well response to conventional therapy.

Scleroderma Associated with Monoclonal Gammopathy of Undetermined Significance

“…Contrary to scleroderma, scleredema spares the distal extremities including hands, fingers, and feet (7-9). Scleromyxedema, a mimicking condition, displays a sclerodermiform papular eruption and histopathologic mucin deposition in the upper and mid dermis (2,10,11,12). Unlike scleredema, it causes fibroblast proliferation and fibrosis (2).…”

“…Unlike scleredema, it causes fibroblast proliferation and fibrosis (2). Similar to scleredema, scleromyxedema is associated with systemic disorders like paraproteinemia (usually Ig) (2,8,11) but not thyroid disease (2,8,10,12).…”

Papular mucinosis: A report of two cases

“…About 20 cases of DPM have been described that were not associated with HCV or HIV infection 8 . Scleroderma (systemic sclerosis), scleredema, eruptive papular xanthoma, lichen amyloidosis, lichen planus and lichenoid drug eruption should be considered in the differential diagnosis of scleromyxedema 1 .…”

“…Mucinoses are a heterogeneous group of disorders in which abnormal amount of mucin accumulates in the skin, either diffusely or locally 1 . Cutaneous mucinoses may be listed as primary, in which mucin deposition is the major histologic property resulting in clinically characteristic lesions, and secondary, in which mucin represents an associated histologic finding.…”

Arndt-Gottron Scleromyxedema: Case Report

“…Mucin is a gel-like substance consi sting of proteins, carbohydrates and acid glucosoaminoglycans present in metabolic disorders and produced by dermal fibroblasts. The etiology and pathogenesis of Arndt-Gottron scleromyxedema have not been fully studied, but it is assumed that the key role in the stimulation of fibroblast proliferation can be played by paraproteinemia, which was detected in 80% of patients, an internal defect of fibroblasts or another not yet identified circulating factor (5,6).…”