title: Farber lipogranulomatosis with predominant joint involvement mimicking juvenile idiopathic arthritis.
abstract: The case of a 10-year-old boy with Farber lipogranulomatosis with predominant joint involvement, subacute, laryngeal and tongue granulomas, microcytic anemia, elevated ESR and CRP, is presented. The boy had no signs of CNS and internal organ involvement. The disease manifested at 6 months; at 11 months the boy had widespread granulomatous polyarthritis with contractures, and juvenile idiopathic arthritis (JIA) was suggested. All antirheumatic therapies failed. Immunologic assessment revealed elevated serum interleukin-1β, increased T-helper, NK and CD25-positive cells, and circulating immune complexes. Our case with predominant rheumatologic manifestations illustrates a differential diagnosis of JIA.
authors: Hospital Pediatric Department, Saint-Petersburg State Pediatric Medical University, Lytovskaya 2, Saint-Petersburg, Russia, 194100, kost-mikhail@yandex.ru.
authors: Mikhail M
authors: MM
authors: Kostik
authors: Irina A
authors: IA
authors: Chikova
authors: Vladislav V
authors: VV
authors: Avramenko
authors: Laly I
authors: LI
authors: Vasyakina
authors: Emmanuelle
authors: E
authors: Le Trionnaire
authors: Vyacheslav G
authors: VG
authors: Chasnyk
authors: Thierry
authors: T
authors: Levade
mesh_terms: D001168:Arthritis / Q000175:diagnosis* / Q000209:etiology*; D001171:Arthritis, Juvenile / Q000175:diagnosis* / Q000209:etiology; D002648:Child; D003937:Diagnosis, Differential; D055577:Farber Lipogranulomatosis / Q000150:complications* / Q000175:diagnosis*; D006801:Humans; D008297:Male
references: Genomics. 1999 Dec 1;62(2):223-31
references: 10610716