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Systemic capillary leak syndrome

M A Barnadas1, A Cisteró, D Sitjas

  • 1Department of Dermatology, Hospital de la Sta. Creu i St. Pau, Barcelona, Spain.

Journal of the American Academy of Dermatology
|February 1, 1995
PubMed
Summary
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Systemic capillary leak syndrome (SCLS) is a rare disorder causing fluid shifts and hypovolemic shock. A case study highlights a patient with IgG kappa paraprotein who experienced recurrent episodes and ultimately succumbed to the condition.

Area of Science:

  • Internal Medicine
  • Hematology
  • Pathology

Background:

  • Systemic capillary leak syndrome (SCLS) is a rare, life-threatening disorder characterized by plasma leakage from blood vessels into surrounding tissues.
  • This condition leads to hypovolemic shock and can have a high mortality rate.
  • The exact etiology of SCLS remains largely unknown.

Observation:

  • A case of SCLS in a 34-year-old woman experiencing recurrent generalized edema and hypovolemic shock is presented.
  • Laboratory findings during acute phases included hypoproteinemia, leukocytosis, elevated hematocrit and hemoglobin, and the detection of a paraprotein IgG kappa chain.

Findings:

  • The patient experienced multiple SCLS episodes despite various therapeutic interventions.
  • The presence of a paraprotein, specifically IgG kappa chain, was noted, which has been frequently observed in SCLS patients.

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  • The patient ultimately succumbed to the disorder 2.5 years after the initial presentation.

    • Implications:

    • This case underscores the severe prognosis of SCLS and the challenges in its management.
    • The recurrent detection of paraprotein IgG kappa chain in this patient suggests a potential link between monoclonal gammopathies and SCLS pathogenesis.
    • Further research into the association between paraproteins and SCLS is warranted to elucidate underlying mechanisms and improve therapeutic strategies.