ISCLS (Clarkson's disease or syndrome)

Q: What is the triad of Idiopathic systemic capillary leak syndrome (ISCLS)?

Answer: Idiopathic systemic capillary leak syndrome (ISCLS) consists of episodes of 

• severe hypotension 
• hypoalbuminemia, and 
• hemoconcentration 

Two important distinctions in this regard are important. First, patients are prone to episodes/attacks of profound derangements of the vascular endothelium with leakage of plasma and proteins into the interstitial compartment. Second, performing differential diagnoses with similar pathologic diseases is extremely important such as:

• Severe sepsis or septic shock 
• Recurrent toxic shock syndrome in women
• Anaphylaxis 
• Anaphylactic attacks of systemic mastocytosis
• Drug reactions
• Hereditary angioedema 

It is also called Clarkson's disease or syndrome.

#vascular

References:

1. CLARKSON B, THOMPSON D, HORWITH M, LUCKEY EH. Cyclical edema and shock due to increased capillary permeability. Am J Med 1960; 29:193.

2. Marks J, Shuster S. Disorders of capillary permeability. Br J Dermatol 1973; 88:619.

3. Gousseff M, Arnaud L, Lambert M, et al. The systemic capillary leak syndrome: a case series of 28 patients from a European registry. Ann Intern Med 2011; 154:464.

4. Druey KM, Parikh SM. Idiopathic systemic capillary leak syndrome (Clarkson disease). J Allergy Clin Immunol 2017; 140:663.