Hyper IgD Syndrome
This autosomal recessive disease is caused by mutations in the MVK gene. Patients develop episodic fevers before the first year of life lasting 3-7 days and recurring every 2-8 weeks. Attacks of fever are associated with skin rash, cervical lymphadenopathy, diarrhea, arthritis/arthralgia, oral ulcerations, abdominal pain, and hepatosplenomegaly. Serum IgD and IgA levels are increased during and between attacks. Amyloidosis can be a serious complication, but it has been reported in only 3% of HIDS patients.