Periodic Fever and Maculopapular Rash

  • Yuriy Stepanovskiy


Periodic fever syndrome with hyper-IgD syndrome (HIDS), is an autosomal recessive autoinflammatory disease characterized by recurrent episodes of fever, abdominal pain, diarrhea, rash, arthralgia, aphthous ulcers, and lymphadenopathy with systemic inflammation

Serum IgD is increased in most patients with HIDS, while more than 20% of them have normal values of serum IgD and almost 50% of patients with other autoinflammatory disorders are identified to have increased levels of serum IgD

The most appropriate laboratory analysis for diagnostic of HIDS is testing of mevalonic acid level in urine during fever episodes


Hyper-IgD syndrome MVK Urine mevalonic acid 


  1. 1.
    Doria A, Zen M, Bettio S, Gatto M, Bassi N, Nalotto L, Ghirardello A, Iaccarino L, Punzi L. Autoinflammation and autoimmunity: bridging the divide. Autoimmun Rev. 2012;12(1):22–30.CrossRefGoogle Scholar
  2. 2.
    Drenth JP, van der Meer JW. Hereditary periodic fever. N Engl J Med. 2001;345(24):1748–57.CrossRefGoogle Scholar
  3. 3.
    Zen M, Gatto M, Domeneghetti M, Palma L, Borella E, Iaccarino L, Punzi L, Doria A. Clinical guidelines and definitions of autoinflammatory diseases: contrasts and comparisons with autoimmunity-a comprehensive review. Clin Rev Allergy Immunol. 2013;45(2):227–35.CrossRefGoogle Scholar
  4. 4.
    Houten SM, Kuis W, Duran M, de Koning TJ, van Royen-Kerkhof A, Romeijn GJ, Frenkel J, Dorland L, de Barse MM, Huijbers WA, Rijkers GT, Waterham HR, Wanders RJ, Poll-The BT. Mutations in MVK, encoding mevalonate kinase, cause hyperimmunoglobulinaemia D and periodic fever syndrome. Nat Genet. 1999;22(2):175–7.CrossRefGoogle Scholar
  5. 5.
    Kuijk LM, Beekman JM, Koster J, Waterham HR, Frenkel J, Coffer PJ. HMG-CoA reductase inhibition induces IL-1beta release through Rac1/PI3K/PKB-dependent caspase-1 activation. Blood. 2008;112(9):3563–73.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Yuriy Stepanovskiy
    • 1
  1. 1.Shoupyk National Medical Academy of Postgraduate EducationKyivUkraine

Personalised recommendations