hyperimmunoglobulinemia D syndrome

hyperimmunoglobulinemia D syndrome
(HIDS), hyperimmunoglobulinemia D with periodic fever syndrome an autoinflammatory disease (q.v.) inherited as an autosomal recessive trait, caused by mutations in the gene for mevalonate kinase, with onset usually before one year of age. It is characterized by attacks of high fever preceded by chills, occurring about every 4 to 8 weeks and lasting 4 to 6 days, often with headache, arthritis and arthralgia, erythematous lesions, and hepatosplenomegaly; serum IgD levels are continuously high. Called also Dutch type periodic fever.

Medical dictionary. 2011.

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