Farber lipogranulomatosis with predominant joint involvement mimicking juvenile idiopathic arthritis.
J Inherit Metab Dis
; 36(6): 1079-80, 2013 Nov.
Article
em En
| MEDLINE
| ID: mdl-23385296
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-1ß, increased T-helper, NK and CD25-positive cells, and circulating immune complexes. Our case with predominant rheumatologic manifestations illustrates a differential diagnosis of JIA.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Artrite
/
Artrite Juvenil
/
Lipogranulomatose de Farber
Tipo de estudo:
Diagnostic_studies
/
Etiology_studies
Limite:
Child
/
Humans
/
Male
Idioma:
En
Revista:
J Inherit Metab Dis
Ano de publicação:
2013
Tipo de documento:
Article