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1: J Am Acad Dermatol. 1995 Feb;32(2 Pt 2):364-6.Links

Systemic capillary leak syndrome.

Barnadas MA, Cisteró A, Sitjas D, Pascual E, Puig X, de Moragas JM.

Department of Dermatology, Hospital de la Sta. Creu i St. Pau, Barcelona, Spain.

Systemic capillary leak syndrome is a rare, severe disorder with a high mortality rate. It consists of the shift of fluid and proteins from the intravascular to the extravascular compartment with subsequent hypovolemic shock. We describe a 34-year-old-woman who had several episodes of generalized edema that evolved to hypovolemic shock. During the acute phase, laboratory investigations revealed marked hypoproteinemia, leukocytosis, and high levels of hematocrit and hemoglobin. A paraprotein IgG kappa chain was detected. Although different therapeutic trials were used, the patient continued to have similar episodes and she died during an acute episode 2 1/2 years after the first symptom of this disorder. The cause of systemic capillary leak syndrome is unknown. The presence of a paraprotein IgG is frequent in this group of patients.

PMID: 7829741 [PubMed - indexed for MEDLINE]

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