Abstract
Background: The idiopathic systemic capillary leak syndrome (ISCLS), also known as Clarkson disease, is an extremely infrequent and commonly misdiagnosed disease. This rare disorder is characterized by paroxysmal capillary hyperpermeability with diffuse severe oedema and hypovolemia, along with hemoconcentration and hypoalbuminemia. Case Summary: A 60-year-old Moroccan man was admitted to our hospital, presenting with a severe hypovolemic shock associated with diffuse ede- matous syndrome following an emotional shock. Laboratory tests showed hemoconcentration, hypoalbuminemia, and a monoclonal gammopathy in the electrophoresis of proteins, while all other laboratory tests were normal. During hospitalization, the patient was conditioned and monitored with careful volume expansion using synthetic colloids and an ascites puncture was performed. After excluding other pathologies, the clinical presenta- tion was consistent with the diagnosis of ISCLS. Recurrent episodes were reported before the patient’s death. Conclusion: The idiopathic systemic capillary leak syndrome is a potentially life threatening disease with a high mortality. The pathophysiology of this condition remains unclear despite all previous attempts at clarification. Knowledge of the limited treatment options is crucial for patients’ prognosis and overall survival. This report aims to raise awareness of ISCLS.
Published Version
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