Case study A possible role for IL-17 in Clarkson's disease Mathieu Simonin a , Pierre Corbeau b , Philippe Durand c , Michelle Rosenzwajg d , Anne Filleron e,f , Tu-Anh Tran e,f,* a Department of Pediatrics, Bic^etre University Hospital, Assistance Publique des H^opitaux de Paris, Le Kremlin Bic^etre, France b Department of Immunology, Nıˆmes University Hospital, Montpellier-Nıˆmes University, Nıˆmes, France c Intensive Care and Resuscitation Unit, Bic^etre University Hospital, Assistance Publique des H^opitaux de Paris, Le Kremlin Bic^etre, France d La Pitie-Salp^etriere Hospital, Biotherapy (CIC-BTi) and Inflammation-Immunopathology-Biotherapy Department (I2B), Assistance Publique des H^opitaux de Paris, Sorbonne Universite, UPMC Univ Paris 06, INSERM, UMRS 959, Immunology-Immunopathology-Immunotherapy (I3), Paris, France e Department of Pediatrics, Nıˆmes University Hospital, Nıˆmes, France f INSERM U 1183, Montpellier University, Montpellier, France article info Article history: Received 5 June 2016 Received in revised form 25 July 2016 Accepted 7 August 2016 Keywords: Children Clarkson's disease IL-17 Systemic capillary leak syndrome abstract Introduction: Systemic capillary leak syndrome (SCLS) is a rare disease characterized by recurrent episodes and a triad of “leak attacks”: hypovolemic shock, generalized edema, hemoconcentration and paradoxical hypoalbuminemia. Case study and discussion: Here we report a case of pediatric idiopathic SCLS with an episode of cough and fever followed two days later by myalgia, livedo, acrocyanosis, and five days later by edema, tachycardia, hypotension, and generalized tonic-clonic seizure. Moreover, we provide evidence for an LPS-induced overproduction of interferon-gamma and interleukin-17 by the patient's peripheral blood mononuclear cells one year after the attack. Conclusion: This observation suggests the involvement of IL-17 in the pathogenesis of this disease. © 2016 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved. 1. Introduction Systemic capillary leak syndrome (SCLS), also known as Clarkson's disease, is a rare and severe condition character- ized by a recurrent stereotypical triad: hypovolemic shock, generalized edema, hemoconcentration and hypo- albuminemia. 1 It is caused by massive fluid extravasation into the interstitial space. Mortality may result from hemodynamic failure in the acute phase or cardiac failure due to reflex circulatory overload in the sub-acute phase. The physiopathology of this disease is still debated. Yet, recent * Corresponding author. Department of Pediatrics, Nıˆmes University Hospital, 1 place Robert Debr e, 30029 Nı ˆmes, France. E-mail address: tu.anh.tran@chu-nimes.fr (T.-A. Tran). Official Journal of the European Paediatric Neurology Society european journal of paediatric neurology 20 (2016) 953 e956 http://dx.doi.org/10.1016/j.ejpn.2016.08.003 1090-3798/© 2016 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.