Hammoudeh (2004) reported a 9-year-old Palestinian boy with Hyper-IgD syndrome. This was the first report of Hyper-IgD syndrome in an Arab patient. The proband presented at 8 months of age with recurrent attacks of fever that reached as high as 40.3°C associated with joint pain and enlarged cervical lymph nodes. He also developed skin rash on several occasions presented as small macules on the chest and one time as a large macule on the dorsum of the left hand and once on the dorsum of the foot. Headache, abdominal pain, nausea, and vomiting were also present in some of the attacks. The initial attacks were occurring as frequently as every 3 weeks, but the interval became larger as the child grew. The diagnosis of familial Mediterranean fever was entertained initially and he was put on colchicines. Laboratory investigation revealed hemoglobin (Hb) as low as 10.3 g/l during the attacks, platelets were within normal limits, WBCs were as high as 24,400, normal iron and total iron-binding capacity (TIBC), erythrocyte sedimentation rate ranged from 40 to 78/h, IgG 622 mg/dl (833-1280), IgA 161 mg/dl (33-202), and IgM 148 mg/dl (48-207). DNA sequence analysis showed that the patient was homozygous for the V3771 mutation in exon 10 of the mevalonate kinase gene, confirming the diagnosis of Hyper-IgD syndrome.

[See: Palestine > Hammoudeh (2004)].