It is possible that the main title of the report Hyper IgD syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.
- hyperimmunoglobulinemia D with periodic fever
Hyper IgD syndrome (HIDS) is a rare inflammatory genetic disorder characterized by periodic episodes or "attacks" of fever associated with additional symptoms including joint pain (arthralgia), skin rash and abdominal pain. Most episodes last several days and occur periodically throughout life. The frequency of episodes and their severity vary greatly from case to case. HIDS is associated with decreased activity of the enzyme mevalonate kinase (MVK). Although many factors can set off a characteristic HIDS episode (e.g., minor infections), most episodes occur without a distinct precipitating event. HIDS is inherited as an autosomal recessive trait.
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