Scleromyxedema is a rare progressive cutaneous mucinosis, usually associated with a systemic involvement and paraproteinemia. Its aetiology remains unknown. The therapeutic options include numerous treatment modalities, however, no standard treatment exists as the rarity of this disease prevents the execution of controlled therapeutic trials. This paper reports a case of a 38-year-old male with...

Scleromyxedema is an infiltrative skin disease produced by hyaluronic acid deposition in the dermis. A benign monoclonal gammopathy is usually present. We report 2 patients with scleromyxedema and systemic illnesses. Both patients had muscle weakness, dysphagia, and weight loss in addition to the skin changes. One also had sclerodactyly, telangiectasias, and Raynaud's phenomenon. Scleromyxedema...

scleromyxedema is a rare, chronic, progressive, fibromucinous disorder of unknown etiology, characterized by lichenoid waxy papules and firm induration of skin of trunk, face, forearm and hands; fibroblast proliferation and mucin deposition in the upper dermis. cutaneous involvement is characteristic but there are several associated systemic manifestations: we observed a case of scleromyxedema ...

Scleroderma-like cutaneous lesions have been found in many pathological conditions and they the clinical appearance of sclerotic or scleroatrophic lesions. Affected skin biopsies described histopathological changes similar to those scleroderma located strictly on systemic sclerosis. These can be inflammatory diseases with autoimmune substrate (generalized morphea, chronic graft versus host dise...

Sir, Lichen myxedematosus (LM) is a rare pathology, described for the ® rst time by Dubreuilh (1) in 1906, characterized by the accumulation of mucinous material in the dermis, with no disorder in the thyroid gland, and usually associated with paraproteinemia. In 1953, Montgomery & Underwood (2) classi® ed LM into 4 clinical types: (1) a generalized lichenoid eruption, later denominated sclerom...

Scleromyxedema is a potentially serious disease that can have various systemic complications. One of the most frequent forms of central nervous system involvement is dermato-neuro syndrome. High-dose intravenous immunoglobulins are among the drug treatments that have been used for this syndrome. We describe 2 patients with scleromyxedema, one of whom developed dermato-neuro syndrome. Both patie...

Many conditions presenting with clinical hard skin and tissue fibrosis can be confused with systemic sclerosis (scleroderma). These disorders have very diverse etiologies and often an unclear pathogenetic mechanism. Distinct clinical characteristics, skin histology, and disease associations may allow one to distinguish these conditions from scleroderma and from each other. A prompt diagnosis is...

Scleromyxedema is a rare chronic cutaneous mucinosis usually associated with a monoclonal gammopathy and underlying systemic disease. The etiology of the disease is not known. There are no standard treatments and response to various therapeutic modalities varies. We report a case of refractory scleromyxedema in a 63-year-old man with a biclonal IgG and IgM λ-gammopathy. The patient was successf...