SCLEROMYXEDEMA WITH MYOPATHY AND HYPERTHYROIDISM

We describe a 62-year-old woman who developed extensive papular skin e ruption with dysphagia and proximal muscle weakness, Laboratory studie s showed a progressive increase of muscle enzymes, lambda monoclonal g ammopathy, and elevated serum thyroid hormones. Several skin and muscl e biopsies were necessary, to reach the correct diagnosis of scleromyx edema in association with hyperthyroidism. Muscle biopsies contained r immed vacuoles with some necrosis and regeneration, but no increased m ucopolysaccharides, Hyperthyroidism was treated without appreciable im provement of the skin and muscle legions. Myopathy is an increasingly recognized feature of scleromyxedema; its pathogenesis is still unexpl ained.