Aagenaes syndrome, also called Lymphedema Cholestasis Syndrome (LSC 1), is a form of idiopathic familial intrahepatic cholestasis associated with lymphedema of the lower extremities. It is named after the Norwegian pediatrician Oyestein Aagenaes, who described the syndrome in 1968. The presence of lymphedema is likely the predisposing factor for development of recurrent infections in such patients.1 Recurrent cellulitis as such has never been described in the literature with Aagenaes syndrome. This case highlights recurrent cellulitis as one of the potential complications of Aagenaes syndrome.
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Der Hagen CB , Eiklid K., Aagenaes O., Freimer NBMapping of locus for cholestasis-lymphoedema syndrome (Aagenaes syndrome) to a 6.6 cM interval on chromosome 15q. Am J Hum Genet.2000;67:994-999.
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Behrman RE, Kliegman RM, Jenson HB, et al. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, PA: Elsevier ; 2007:1670.
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Aagenaes O., Mebdo S.Hereditary intrahepatic cholestasis with lymphoedema-Aagenaes syndrome. Tidsskr Nor Laegeforen. 1993;113:3673-3677.
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Drivdal M., Trydal T., Hagve TA, Bergstad I., Aagenaes O.Prognosis, with evaluation of general biochemistry, of liver disease in lymphoedema cholestasis syndrome 1 (LCS 1/Aagenaes syndrome). Scand J Gastroenterol. 2006;41:465-471.
