Idiopathic Systemic Capillary Leak Syndrome: Cutaneous Involvement Can Be MisleadingFardet L. · Kerob D. · Rybojad M. · Vignon-Pennamen M.D. · Schlemmer B. · Guermazi A. · Morel P. · Lebbé C.
Departments of aDermatology, bPathology and cIntensive Care, Centre Hospitalier Universitaire Saint-Louis, Paris, France; dDepartment of Radiology, University of California, San Francisco, Calif., USA
Background: Systemic capillary leak syndrome (SCLS) is a severe disorder characterized by unexplained rapid transfer of considerable volumes of plasma from the intravascular to the extravascular compartment. For some cases of SCLS, no aetiology is evident and these cases are reported as idiopathic (ISCLS). Objectives: To describe the cutaneous findings in 3 patients with ISCLS. Results: Cutaneous involvement consisted in sclerosis, livedo, purpura and photodistributed maculopapular erythematous rash. Dermal mucinosis was proven by biopsy in 1 patient. No underlying disease was diagnosed during follow-up. Conclusion: The above-mentioned cutaneous findings can be present during acute attacks of ISCLS. They seem specifically related to the ISCLS and not indicative of an underlying disease.
Prof. Céleste Lebbé, MD, PhD
Service de dermatologie du professeur P. Morel
Centre Hospitalier Universitaire Saint-Louis, 1, avenue Claude-Vellefaux
FR–75010 Paris (France)
Tel./Fax +33 1 42 49 95 90, E-Mail email@example.com
Received: February 26, 2004
Accepted: June 18, 2004
Number of Print Pages : 5
Number of Figures : 3, Number of Tables : 1, Number of References : 30
Dermatology (International Journal for Clinical and Investigative Dermatology)
Vol. 209, No. 4, Year 2004 (Cover Date: 2004)
Journal Editor: J.-H. Saurat, Geneva.
ISSN: 1018–8665 (print), 1421–9832 (Online)
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