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Scleromyxedema

Author
Franco Rongioletti, MD
Section Editor
Jeffrey Callen, MD, FACP, FAAD
Deputy Editor
Abena O Ofori, MD

INTRODUCTION

Scleromyxedema, also known as generalized and sclerodermoid lichen myxedematosus or Arndt-Gottron disease, is a primary cutaneous mucinosis characterized by a generalized papular and sclerodermoid cutaneous eruption that usually occurs in association with monoclonal gammopathy. Affected patients develop numerous waxy firm papules and plaques that demonstrate mucin deposition, increased fibroblast proliferation, and fibrosis on histologic examination. Systemic manifestations may involve the cardiovascular, gastrointestinal, pulmonary, musculoskeletal, renal, or nervous systems, and may lead to significant morbidity and mortality.

Scleromyxedema should be distinguished from localized lichen myxedematosus, a form of lichen myxedematosus that presents with waxy, firm papules and plaques involving limited areas. Unlike scleromyxedema, sclerotic features, systemic involvement, and monoclonal gammopathy are absent in localized lichen myxedematosus. Scleroderma and scleredema are additional disorders that present with sclerodermoid features but are unrelated to scleromyxedema. Scleromyxedema also is distinct from myxedema of thyroid disease. (See "Overview of the clinical manifestations of systemic sclerosis (scleroderma) in adults" and "Scleredema" and "Clinical manifestations of hypothyroidism", section on 'Skin'.)

The clinical features, diagnosis, and management of scleromyxedema will be reviewed here.

EPIDEMIOLOGY

Scleromyxedema is a rare disease that usually affects middle-aged adults between the ages of 30 and 80 years with no race or sex predominance [1]. In a multicenter retrospective study of 30 patients with scleromyxedema, the mean age of affected patients was 59 years [1]. This illness has not been reported in infants and young children.

PATHOGENESIS

The pathogenesis of scleromyxedema is unknown. The significance of the associated monoclonal gammopathy and the underlying plasma cell clone is debated. The main hypothesis is that circulating cytokines such as interleukin (IL)-1, TNF-alpha, and TGF-beta, known to stimulate glycosaminoglycan synthesis and fibroblast proliferation in the skin, could play a role [2-4]. Clinical remission of scleromyxedema following autologous stem cell transplantation suggests that the bone marrow may be a source of these circulating factors [2,5].

                            

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Literature review current through: Nov 2016. | This topic last updated: Thu Jul 30 00:00:00 GMT+00:00 2015.
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References
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