[Hypergammaglobulinemia D syndrome].
The hyperimmunoglobulinemia D syndrome is characterized by early onset of attacks of periodic fever and an elevated serum polyclonal Ig D (> 100 U/ml). Symptoms during attacks include joint involvements (arthralgia/arthritis), skin lesions, swollen lymph nodes, headache and abdominal complaints (vomiting, diarrhea and pain). The ethiopathogenia is unknown. It is transmitted by autosomal recessive inheritance. The hyperimmunoglobulinemia D syndrome should be distinguished from other periodic febrile syndroms such as systemic-onset juvenile rheumatoid arthritis, CINCA syndrome, FADA syndrome, familial mediterranean fever and adult-onset Still disease. There is no therapy for the syndrome but the prognosis is good because the frequency and severity of the attacks tends to diaeminish with age.
Medrano San Ildefonso
M
M
Servicio de Reumatología, Nueva Clínica Quirón, Zaragoza.
Bruscas Izu
C
C
D003937:Diagnosis, Differential; D006801:Humans; D006942:Hypergammaglobulinemia / Q000150:complications / Q000175:diagnosis / Q000209:etiology; D007072:Immunoglobulin D
D007072:Immunoglobulin D