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 |
