Idiopathic capillary leak syndrome complicated by massive rhabdomyolysis.

[systemic capillary leak syndrome]

Idiopathic systemic capillary leak syndrome (Clarkson 's disease ) is characterized by recurring attacks of increased capillary permeability , resulting in severe hypovolemic shock due to plasma extravasation . Additional laboratory features include association with a monoclonal gammopathy , extreme hemoconcentration , and hypoalbuminemia . Rare manifestations of this syndrome are renal damage and rhabdomyolysis due to increased compartment pressure and ischemic myonecrosis . We present the findings in two patients with capillary leak syndrome complicated by severe rhabdomyolysis , in one case leading to acute renal failure . We review therapeutic aspects of this rare syndrome and emphasize the importance of early diagnosis and of prompt and aggressive fluid replacement .