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Disseminated lipogranulomatosis
1Department of Pediatric Medicine and Department of Orthopedics, IPGMER and SSKM Hospital, Kolkata, India.
Indian Pediatrics
|February 27, 2009
Abstract:
Farber disease or disseminated lipogranulomatosis is a rare inherited disorder of lipid metabolism resulting from a defect in ceramide degradation. Because of the feature of nodular swellings around various joints, this may sometimes be confused with juvenile idiopathic arthritis. We report a 4-year-old boy with Farber disease who presented with nodular swellings around the joint, angle of the mouth and conjunctiva, and was subsequently diagnosed to be a case of Farber Disease.
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