Capillary leak syndrome
Synonym(s)
Clarkson disease; Clarkson's syndrome
HistoryThis section has been translated automatically.
Clarkson, 1960
DefinitionThis section has been translated automatically.
Discharge of blood, lymphatic fluid and proteins from capillaries with circumscribed or diffuse distribution in the tissue. Characteristic are hypotension, hypoalbuminemia without albuminuria and generalized edema.
EtiopathogenesisThis section has been translated automatically.
- Caused by generalized capillary hyperpermeability.
- Pathogenetic association with monoclonal gammopathy is discussed by some authors.
- In case reports, isolated drugs have been described as triggering agents (e.g. acitretin, interleukins, imiquimod).
ManifestationThis section has been translated automatically.
Men and women are equally affected.
Clinical featuresThis section has been translated automatically.
Course in several days: Between day 1-4 abdominal discomfort, nausea, generalized edema, hypotension, cardiopulmonary dysregulation, acute renal failure (partly caused by hypovolemia). Afterwards counterregulation of extravasation and occurrence of pulmonary edema.
Complication(s)This section has been translated automatically.
The disease can lead to potentially life-threatening hypotension and multiple organ failure.
TherapyThis section has been translated automatically.
Symptomatic therapy.
Progression/forecastThis section has been translated automatically.
A similar, sporadically occurring clinical picture is described as episodic angioedema with eosinophilia.
LiteratureThis section has been translated automatically.
Duron L et al (2014) Idiopathic and secondary capillary leak syndromes: A systematic review of the literature. Rev Med Interne doi: 10.1016/j
- Vos LE et al (2007) Acitretin induces capillary leak syndrome in a patient with pustular psoriasis. J Am Acad Dermatol 56: 339-342
Xie Z et al (2014) Inflammatory Markers of the Systemic Capillary Leak Syndrome (Clarkson Disease). J Clin Cell Immunol PubMed PMID: 25405070
- Zancanaro A et al (2015) Clinical and pathological findings of a fatal systemic capillary leak syndrome (clarkson disease): a case report. Medicine (Baltimore) 94:e591