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 .

Diseases
Validation

Diseases presenting "edema" symptom

acute rheumatic fever

adrenomyeloneuropathy

aniridia

cadasil

canavan disease

classical phenylketonuria

coats disease

congenital toxoplasmosis

cushing syndrome

cutaneous mastocytosis

erythropoietic protoporphyria

esophageal carcinoma

fabry disease

familial mediterranean fever

focal myositis

harlequin ichthyosis

homocystinuria without methylmalonic aciduria

inclusion body myositis

lamellar ichthyosis

liposarcoma

lymphangioleiomyomatosis

malignant atrophic papulosis

pleomorphic liposarcoma

systemic capillary leak syndrome

trochlear dysplasia

von hippel-lindau disease

waldenstrÃ¶m macroglobulinemia

This symptom has already been validated