Scleromyxedema is a sclerotic variant of papular mucinosis, in which lichen
oid papules and scleroderma-like features are present. We describe a patien
t with scleromyxedema with IgG type lambda chain paraprotein, a systemic sc
lerosis-like illness, and myositis. The patient's serum contained Sd 70 ant
ibodies, characteristic of scleroderma. Electromyography showed signs of ac
ute myositis and the creatine phosphokinase (CPK) level was elevated. Multi
ply passaged fibroblasts from the patient's skin lesions showed altered gro
wth response in vitro. The patient was treated with cyclosporin (4 mg/kg/da
y) with improvement.