Systemic capillary leak syndrome

• Published in: Journal of the American Academy of Dermatology Vol. 32; no. 2; pp. 364 - 366
• Main Authors: Barnadas, María A, Cisteró, Anna, Sitjas, Dolors, Pascual, Enric, Puig, Xavier, de Moragas, JoséM
• Format: Journal Article
• Language: English
• Published: New York, NY Mosby, Inc 01.02.1995; Elsevier
• Subjects: Adult; Allergic diseases; Biological and medical sciences; Capillary Permeability; Edema - etiology; Fatal Outcome; Female; Hematocrit; Hemoglobins - analysis; Humans; Hypoproteinemia - etiology; Hypotension - etiology; Immunoglobulin G - analysis; Immunoglobulin kappa-Chains - analysis; Immunopathology; Leukocytosis - etiology; Medical sciences; Paraproteins - analysis; Shock - etiology; Skin allergic diseases. Stinging insect allergies; Syndrome; Vascular disease; Case study; Human; Systemic capillary leak syndrome; Cardiovascular disease; Female; Blood capillary; Permeability; Clarkson disease; Generalized
• ISSN: 0190-9622; 1097-6787
• DOI: 10.1016/0190-9622(95)90405-0

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 κ 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.