A case of scleromyxedema without monoclonal gammopathy responded to treatment with IVIG

Lichen myxedematosus’’ is a primary inflammatory dermatosis characterized by fibroblast proliferation and mucin deposition in the skin. Scleromyxedema is the most frequent variant of lichen myxedematosus and is accompanied by papular lesions, diffuse sclerosis, and erythema. It is usually associated with paraproteinemias. Scleromyxedema without monoclonal gammopathy is a rare and atypical variant of lichen myxedematosus. Systemic involvement and sometimes fatal course can be seen with scleromyxedema. Although several treatment modalities have been tried as case reports for the disease, there isn’t any definitive treatment yet. Intravenous immunoglobulin’s mechanism of action in scleromyxedema is still not clear as well as etiopathogenesis of scleromyxedema. Herein we describe a scleromyxedema case without monoclonal gammopathy and his response to the treatment with intravenous immunoglobulin.