[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.