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Genetic and Rare Diseases Information Center (GARD)

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Hyper-IgD syndrome

Other Names for this Disease
  • Hyper IgD syndrome
  • Hyperimmunoglobulinemia D and periodic fever syndrome
  • Periodic fever Dutch type
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What are the signs and symptoms of hyper IgD syndrome?

Hyper IgD syndrome is characterized by periodic high fevers accompanied by lymphadenopathy, abdominal pain, diarrhea, headache, joint pain, hepatomegaly and/or splenomegaly, and skin lesions.[1][2] Most episodes last several days and occur periodically throughout life.[3] The frequency of episodes and their severity vary greatly from case to case.[3][1] The first attack usually takes place during infancy.[2] Patients may have no symptoms between attacks. However, in some patients, the attacks may be so frequent that the symptoms persist.[1]
Last updated: 3/5/2013

  1. Shinawi M, Scaglia F. Hereditary Periodic Fever Syndromes. Medscape Reference. 2013; Accessed 3/5/2013.
  2. Frenkel J, Simon A. Hyperimmunoglobulinemia D with recurrent fever. Orphanet. 2011; Accessed 3/5/2013.
  3. Hyper IgD Syndrome. National Organization for Rare Disorders (NORD). 2006; Accessed 3/5/2013.