An unusual case of swelling--Clarkson's syndrome.

[systemic capillary leak syndrome]

Clarkson 's syndrome , also called idiopathic systemic capillary leak syndrome is a rare condition characterised by vascular hyper permeability resulting in extreme intravascular volume depletion . The syndrome is unique and almost paradoxical in its presentation , with findings initially suggesting overwhelming heart failure , but in reality the extra vascular fluid represents overt capillary leak , with ultimate intravascular volume depletion , a low output state and hypovolemic shock . Previously described characteristics have classically included severe oedema and anasarca with rapid , profound shock , typically accompanied by haemoconcentration . The authors describe a patient , initially seeming benign in presentation , who rapidly progressed with confusing findings of fluid overload by examination and imaging , ultimately manifesting these findings by severe capillary leak rather than hydrostatic oedema , with ultimate hypovolaemic shock , multisystem organ failure and death . Our aim is that by describing clinical , haemodynamic and pathologic descriptors of the disease , the authors can aid in increasing physician awareness of this unusual syndrome .

Diseases
Validation

Diseases presenting "classically included severe oedema" symptom

systemic capillary leak syndrome

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