Idiopathic systemic capillary leak syndrome: cutaneous involvement can be misleading.

[systemic capillary leak syndrome]

Systemic capillary leak syndrome (SCLS ) is a severe disorder characterized by unexplained rapid transfer of considerable volumes of plasma from the intravascular to the extravascular compartment . For some cases of SCLS , no aetiology is evident and these cases are reported as idiopathic (ISCLS ).To describe the cutaneous findings in 3 patients with ISCLS .Cutaneous involvement consisted in sclerosis , livedo , purpura and photodistributed maculopapular erythematous rash . Dermal mucinosis was proven by biopsy in 1 patient . No underlying disease was diagnosed during follow-up .The above-mentioned cutaneous findings can be present during acute attacks of ISCLS . They seem specifically related to the ISCLS and not indicative of an underlying disease .