[Idiopathic systemic capillary leak syndrome (Clarkson disease) : A rare cause of recurrent life-threatening edema]

Abstract

This case report describes the episodic occurrence of severe generalized edema in a young female patient, who developed hypertension with a massive hemoconcentration (hematocrit >0.5, hemoglobin >20g/dl) and hypoalbuminemia during the course of these acute disease phases. After the first two disease exacerbations were overcome, there was a complete regression of symptoms. After a long symptom-free interval, a new exacerbation occurred as a result of which critical organ ischemia occurred due to the severe hypotension and massive edema. Despite all treatment measures a severe compartment syndrome of the lower extemities with subsequent rhabdomyelosis developed. The patient ultimately died as a result of treatment-refractory cardiovascular failure. The idiopathic systemic capillary leak syndrome (SCLS, also known as Clarkson disease) is a rare and potentially life-threatening disease with a high mortality. Since the first description of the disease only approximately 500 cases have been published worldwide. The pathophysiology of this disease remains unclear despite all previous attempts at clarification. Regulation processes of endothelial permeability seem to be essentially disturbed. Affected patients have a monoclonal gammopathy of undetermined signficance conspicuously often; however, the knowledge of the limited treatment options is of fundamental importance for the prognosis and overall survival of patients.

Keywords: Edema, generalized; Hyperpermeability, vascular, endothelial; Hypotension; Hypovolemic shock; Monoclonal gammopathy of undetermined significance.