Scleromyxedema without paraproteinemia

Abstract

Background: Scleromyxedema is a rare generalized form of lichen myxedematosus, a chronic cutaneous mucinosis of unknown etiology usually associated with a monoclonal gammopathy and underlying systemic disorders. It is characterized by the presence of lichenoid papules and diffuse indurations of the skin. Histologically, mucin deposits are observed in the dermis as variable degrees of fibrosis. Numerous treatment modalities have been reported as producing partial or inconsistent responses associated with significant adverse effects.

Methods: We report an unusual case of scleromyxedema not associated with monoclonal gammopathy in a young patient who was treated with thalidomide.

Results: Patient remained stable with maintenance of injuries despite treatment with thalidomide.

Conclusions: Scleromyxedema is a rare presentation for which a defined therapeutic regimen remains to be established. Treatment with thalidomide has proved to be effective in the management of these patients. We suggest that these patients should be followed up with periodic protein electrophoresis with immunofixation for a monoclonal component in blood and urine. As the therapeutic approach to scleromyxedema remains challenging and treatment is based on symptomatic presentation, further clinical studies to substantiate an effective therapeutic regimen with a positive long-term safety and risk profile are required.