Idiopathic systemic capillary leak syndrome (SCLS): case report and systematic review of cases reported in the last 16 years

Abstract

'Idiopathic systemic capillary leak syndrome (SCLS), or Clarkson's disease is an unusual entity first described 45 years ago. It presents with recurrent episodes of shock due to leakage of the plasma, which is reflected by accompanying hemo-concentration, hypo-albuminemia and edema. We report the case of a young man with multiple episodes of shock with generalized edema and pleural effusion. An initial diagnosis of sepsis was made, but the lack of fever and absence of a demonstrable organism or source led to a revision of diagnosis to SLCS. The last review of this syndrome which analyzed 24 cases was published in 1990. Since then, 75 more cases have been reported. This article reviews these cases.