Capillary leak syndrome

Author: Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 29.10.2020

Synonym(s)

Clarkson disease; Clarkson's syndrome

History
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Clarkson, 1960

Definition
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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.

Etiopathogenesis
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  • 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).

Manifestation
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Men and women are equally affected.

Clinical features
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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)
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The disease can lead to potentially life-threatening hypotension and multiple organ failure.

Therapy
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Symptomatic therapy.

Progression/forecast
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A similar, sporadically occurring clinical picture is described as episodic angioedema with eosinophilia.

Literature
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  1. Duron L et al (2014) Idiopathic and secondary capillary leak syndromes: A systematic review of the literature. Rev Med Interne doi: 10.1016/j

  2. Vos LE et al (2007) Acitretin induces capillary leak syndrome in a patient with pustular psoriasis. J Am Acad Dermatol 56: 339-342
  3. Xie Z et al (2014) Inflammatory Markers of the Systemic Capillary Leak Syndrome (Clarkson Disease). J Clin Cell Immunol PubMed PMID: 25405070

  4. 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

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020