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Systemic capillary leak syndrome is a rare disorder, first described in 1960, characterized by recurrent episodes of shock, hemoconcentration, and hypoalbuminemia. Diagnosis is primarily clinical, and management includes supportive care and immunoglobulin therapy. Given its potentially life-threatening course, early recognition and timely treatment are essential to improving outcomes. We report the case of a 48-year-old man with recurrent episodes of shock, hemoconcentration, and hypoalbuminemia, initially misdiagnosed as sepsis. He required intensive care management and underwent fasciotomies of all four extremities due to compartment syndrome. During the second hospitalization, he was correctly diagnosed with systemic capillary leak syndrome. Treatment with intravenous immunoglobulins, theophylline, and salbutamol was initiated, leading to a sustained favorable clinical response to date.