Abstract
A 46 year old female was admitted for evaluation of paresthesia and weakness of extremities and pretibial edema. She was diagnosed as having Crow-Fukase syndrome because she had cutaneous hemangioma, hypertrichosis, hypothyroidism, general edema, ascites, pericardial effusion, polyneuropathy, splenomegaly and IgA-λ type M-protein. Twenty-five days after admission anasarca worsened and renal function declined rapidly. Thirtyfive days after admission hemodialysis was initiated to manage anasarca and renal function. The results of blood examination at the beginning of hemodialysis were as follows; BUN; 135mg/dl Cr; 4.5mg/dl, K; 6.6mEq/l Corticosteroid therapy was also started. Three days after the initiation of hemodialysis, urine volume began recovering rapidly. Corticosteroid therapy, including pulse therapy, was effective in the recovery of renal function and in reducing other symptoms. Percutaneous renal needle biopsy was performed when serum creatinine had returned to the normal level. Renal histology showed thickening of the glomerular capillary wall and an expanded subendothelial area with deposition of amorphous materials. Crow-Fukase syndrome is a rare disease characterized by general edema. Although renal dysfunction was noticed recently the degree of renal impairment is usually modest. We report here a case of this syndrome who had severe renal dysfunction and who was treated successfully with hemodialysis and corticosteroids.
