A Rare Presentation of Plasma Cell Disorders - Scleredema: Case Report

Background: Scleredema is a rare connective tissue disorder characterized by thickening of the skin and subcutaneous tissue, involving skin of the neck, shoulders, upper limbs and face. Its association with paraproteinemia is very rare. Case Presentation: A 24year -old female homemaker presented with progressive skin tightening over her upper limbs, face, back, and chest, along with difficulty swallowing for three years. Examination revealed taut, shiny facial skin with decreased wrinkling, reduced mouth opening and skin tightening extending to the arms. She did not have other symptoms suggestive of systemic sclerosis. Rheumatological workup was negative for systemic sclerosis. Skin biopsy showed collagen fibers deposition separated by clear spaces suggestive of scleredema. Further workup revealed serum electrophoresis suggestive of an IgA lambda peak. Her bone marrow biopsy revealed cellular marrow with approximately 20% plasmacytosis. Flowcytometry found 20% plasma cells in the interstitium with immunophenotypes of CD 138+, confirming the diagnosis of Smoldering Multiple Myeloma. Conclusion: Scleredema is a rare disorder which has an unexplained association with paraproteinemia.