Retinopathy in a Case of Farber's Lipogranulomatosis

A syndrome characterized by progressive hoarseness, nodular swellings over the joints simulating rheumatoid arthritis, and death in early infancy was described by Farber and colleagues in 1957 under the heading of "disseminated lipogranulomatosis."1It is currently being reviewed by Crocker et al.2The entity has come to be known as Farber's disease since the term "lipogranulomatosis" is not sufficiently distinctive and since Farber had established priority by referring to it in previous discussions of the lipidoses.3 One of Farber's original three cases was noted to have been blind, but the fundi showed no abnormality and no histologic study was made of the eyes postmortem. In the other two cases of the entity which have been reported,4,5no mention has been made of any ocular abnormality in the retina or choroid. The present report is based on the clinical and pathologic study of a patient who was