단세포군 감마글로불린병증을 동반한 경화점액수종

Abstract

Scleromyxedema is a rare disorder characterized by generalized papular and sclerodermoid eruption, increased fibroblast proliferation, mucin deposition, and monoclonal gammopathy in the absence of thyroid disease. It is a generalized subtype of lichen myxedematosus. A paraproteinemia, typically an IgG λ, is observed in more than 80% of patients with scleromyxedema. Here, we report a 38-year-old woman with a 1-year history of a progressively spreading of eruption of small papules on the entire body, including the face, neck, arms, legs, and trunk. Laboratory tests were within normal limits, except λ light chain monoclonal gammopathy. We administered oral retinoid and topical steroid with slight clinical improvement. To our knowledge, this is the first reported case of scleromyxedema in Korea. (Korean J Dermatol 2011;49(5):440∼443)