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Idiopathic Systemic Capillary Leak Syndrome: Cutaneous Involvement Can Be Misleading

Fardet L.a · Kerob D.a · Rybojad M.a · Vignon-Pennamen M.D.b · Schlemmer B.c · Guermazi A.d · Morel P.a · Lebbé C.a
Departments of aDermatology, bPathology and cIntensive Care, Centre Hospitalier Universitaire Saint-Louis, Paris, France; dDepartment of Radiology, University of California, San Francisco, Calif., USA Dermatology 2004;209:291–295 (DOI:10.1159/000080851)


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.


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