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December 1971

Picture of the Month

Author Affiliations

Boston; Philadelphia
From the Department of Pediatrics, Temple University Health Sciences Center, Philadelphia.

Am J Dis Child. 1971;122(6):513-514. doi:10.1001/archpedi.1971.02110060083014

Denouement and Discussion 

Farber's Disease (Lipogranulomatosis) 

Manifestations  The major manifestations include nodular masses mainly over the wrists and ankles, joint swellings and restriction of movement, mental retardation, and marked failure to thrive. Death usually occurs by 2 years of age. Other manifestations consist of recurrent infections, weak and hoarse cry, noisy breathing, hyperirritability, dyspnea, hepatomegaly, flesh colored papular eruptions, xanthoma-like lesions on the face and hands, and hyporeflexia. Histiocytes, lymphocytes, fibroblasts, and foam cells are seen on microscopic examination of the nodules. An increase in the ceramide level and ganglioside content of these tissues has also been reported.

Genetics  At the present time the condition appears to be inherited as an autosomal recessive trait.

Treatment  The majority of the patients die by 2 years of age. Various types of treatment have been used including corticosteroids and radiotherapy but these have been of no obvious benefit.

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