Scleromyxedema: A case report and review of the literature

Abstract

Scleromyxedema (SM) is a sclerotic variant of lichen or papular mucinosis in which lichenoid papules and scleroderma-like features are both present. It is a rare deposition disorder characterized by generalized papular and sclerodermoid eruptions, mucin deposition, increased fibroblast proliferation, fibrosis, and monoclonal gammopathy (also known as paraproteinemia) mainly of the immunoglobulin G-lambda type in the absence of thyroid disease. It usually affects middle-aged adults and shows no gender or racial predilection. In addition to the skin findings and paraproteinemia, patients with SM have variable multisystem affections that mimic systemic sclerosis; the systems which are commonly involved include the gastrointestinal tract, musculoskeletal, pulmonary, cardiovascular, renal, and central nervous systems, leading to significant morbidity and mortality. Prominent symptoms include dysphagia, proximal muscle weakness, and dyspnea on exertion; less common but important findings include central nervous system involvement in the form of encephalopathy, convulsions, coma, and psychosis.