[Juvenile Crow-Fukase syndrome with response to bolus of methylprednisolone after failure of treatment by plasma exchange].

A 20-year-old woman was hospitalized because of abdominal distention. She had developed facial edema about one year earlier, and recently amenorrhea and red verrucae on the chest and abdomen. Neurological examination disclosed hypesthesia, paresthesia, and diminished tendon reflexes in the arms and legs. The level of serum immunoglobulin A (IgA) was elevated and an M protein was detected. Examination of the bone marrow disclosed abnormal increase in plasma cells. Results of glucose tolerance test were mildly abnormal. The patient was diagnosed as Crow-Fukase syndrome. Plasma exchange was done four times and melphalan was given orally for two weeks, but the level of serum IgA increased further. Then one bolus injection of methylprednisolone decreased the serum IgA with improvement in other signs. The disorder is now controlled satisfactory with a low dose of prednisolone.