A case report of systemic capillary leak syndrome (Clarkson's disease).

[systemic capillary leak syndrome]

Systemic capillary leak syndrome (SCLS ) is a very rare disorder also known as Clarkson 's disease . The condition is characterized by recurrent episodes of severe capillary hyperpermeability resulting in severe hemoconcentration , hypoalbuminemia , hypovolemia and shock . We describe a 41 -year -old previously healthy man who was admitted to hospital on several occasions with rapidly developing hypovolemic shock accompanied by extreme hemoconcentration and hypoalbuminemia . Our case is similar to other reports describing patients with SCLS where the initial suspicions have been pointing towards septic shock . He received a combination of prophylactic treatment with theophylline , beta -agonists , immunoglobulins and statins but eventually died after a severe episode of SCLS that ended with recurrent cardiac arrest . Clinical autopsy revealed pulmonary edema and acute and chronical organic fluid overload . SCLS should be kept in mind when treating patients suffering from attacks of severe idiopathic edema and mimics recurrent septic shock where no pathogen is found . The pathogenesis is unknown and the attacks may be lethal .

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Diseases presenting "mimics recurrent septic shock where no pathogen is found" symptom

systemic capillary leak syndrome

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