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

[systemic capillary leak syndrome]

'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 .