Hyper IgD syndrome
also see Fever, Periodic

• a periodic fever syndrome
• Occurs in infancy; life-long persistence of recurrent attacks
• Last 3-7 days; irregular intervals
• Adenitis, abdominal pain, diarrhea, arthralgia, rash
• Elevated IgD >100 U/ml is considered diagnostic (but can be normal); leukocytosis and elevations of acute-phase reactants during attacks
• Autosomal recessive; due to defect in cholesterol synthesis
• Supportive treatment

CHLA Board Review 2005