Recurrent Cellulitis in a Case of Aagenaes Syndrome

Abstract

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.