PSEUDO PERIODIC FEVER WITH HYPERIMMUNOGLO BULINEMIA D - A NEVER-ENDING STORY WITH A PROBABLE ANTENATAL ONSET

Diagnosis of inflammatory non-infectious diseases with a neonatal onse t is often retrospective. it may lead to aggressive and iatrogenic pro cedures. Patient. - A 6-year-old boy was suffering, since birth, from recurrent febrile attacks including rashes, gastrointestinal manifesta tions and inflammatory joint involvement. This syndrome, partially imp roved with steroids, could have been of antenatal onset. Since the age of 4 years, the patient is considered as having hyper-IgD syndrome (H IDS). Discussion. - HIDS must be distinguished from familial Mediterra nean fever. Patients suffer from recurrent fever concomitant to inflam matory joint involvement, abdominal distress, skin lesions, swollen ly mph nodes and hepatosplenomegaly (especially seen in children). All pa tients have high serum IgD (> 100 UI/mL) and IgA levels. Nevertheless, a high IgD level is not specific. Our case could also be part of the CINCA (chronic, infantile, neurological, cutaneous and articular) synd rome, which includes similar early manifestations associated with a co nstant neurological and frequent ophthalmological involvement and epip hyseal changes; to dale, these last three manifestations are nor prese nt in our patient. Conclusion. - HIDS and CINCA syndrome are not known to be modified by any effective therapeutic agent. When presenting at birth, these inflammatory diseases must be considered as entities wit h a rarely described potential severity. (C) 1998 Elsevier, Paris.