Scleromyxedema: A potentially disabling and fatal disease

Scleromyxedema (SCL), considered to be the generalized and sclerodermoid subtype of lichen myxedematosus (papular mucinosis), is a disease with a chronic, progressive, and potentially disabling course. It is usually, although not invariably, associated with a monoclonal gammopathy which rarely leads to multiple myeloma and with many systemic manifestations. The therapy is difficult and the prognosis is guarded. The author, on the basis of literature and personal experience, review the anatomo-clinical manifestations of this uncommon disease.