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


Turan A., AYDOĞAN K., BALABAN ADIM Ş., Turan H., SARICAOĞLU H.

TURKDERM-ARCHIVES OF THE TURKISH DERMATOLOGY AND VENEROLOGY, cilt.46, sa.4, ss.223-225, 2012 (SCI-Expanded) identifier identifier

Özet

Lichen myxedematosus is a primary inflammatory dermatosis characterized by fibroblast proliferation and mucin deposition in the skin. Scleromyxedema is the generalized 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, there is no definitive treatment available for the disease. Satisfactory results have been obtained in the case reports in which intravenous immunoglobulin treatment was used.The mechanism of action of intravenous immunoglobulin in scleromyxedema is still not clear as well as the etiopathogenesis of scleromyxedema. Herein, we describe the case of a patient with scleromyxedema without monoclonal gammopathy and his response to the treatment with intravenous immunoglobulin. (Turkderm 2012; 46: 223-5)