Pediatric Nephrology

, Volume 21, Issue 8, pp 1200–1205 | Cite as

Hyper-IgE syndrome and autoimmunity in Mexican children

  • Marco Yamazaki-NakashimadaEmail author
  • Samuel Zaltzman-Girshevich
  • Silvestre Garcia de la Puente
  • Beatriz De Leon-Bojorge
  • Sara Espinosa-Padilla
  • Marimar Saez-de-Ocariz
  • Daniel Carrasco-Daza
  • Victor Hernandez-Bautista
  • Lorenzo Pérez-Fernandez
  • Francisco Espinosa-Rosales
Brief Report


Hyper-IgE syndrome (HIES) is a primary immunodeficiency characterized by recurrent skin abscesses, recurrent pneumonia with pneumatocele formation, eczema, eosinophilia, and elevated levels of serum IgE. Patients with the autosomal recessive (AR) form of HIES appear to be prone to developing autoimmune diseases. We present two cases of HIES with autoimmune complications; one case was a product of a consanguineous marriage, the other one was a sporadic case. The first patient presented with recurrent episodes of erythema nodosum, warts, bronchiolitis obliterans and thrombocytopenia. The second patient developed glomerulonephritis resulting in endstage renal failure. She later developed malar rash, oral ulcers, cerebral infarcts with vasculitis and positive ANA, anti-dsDNA, and antiphospholipid antibodies. We discuss the dilemma in treating patients who present with both primary immunodeficiency and autoimmunity.


Systemic lupus erythematosus Antiphospholipid syndrome Hyper-IgE syndrome Autoimmunity Primary immunodeficiency CNS vasculitis 



The authors thank Dr. Joann Lin from the department of Pediatrics, Division of Allergy, Immunology and Rheumatology at UCLA and an anonymous reviewer for helpful discussions.


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Copyright information

© IPNA 2006

Authors and Affiliations

  • Marco Yamazaki-Nakashimada
    • 1
    Email author
  • Samuel Zaltzman-Girshevich
    • 2
  • Silvestre Garcia de la Puente
    • 2
  • Beatriz De Leon-Bojorge
    • 3
  • Sara Espinosa-Padilla
    • 1
  • Marimar Saez-de-Ocariz
    • 4
  • Daniel Carrasco-Daza
    • 3
  • Victor Hernandez-Bautista
    • 1
  • Lorenzo Pérez-Fernandez
    • 5
  • Francisco Espinosa-Rosales
    • 1
  1. 1.Department of Clinical ImmunologyInstituto Nacional de PediatríaMexico CityMexico
  2. 2.Department of NephrologyInstituto Nacional de PediatríaMexico CityMexico
  3. 3.Department of PathologyInstituto Nacional de PediatríaMexico CityMexico
  4. 4.Department of DermatologyInstituto Nacional de PediatríaMexico CityMexico
  5. 5.Department of PulmonologyInstituto Nacional de PediatríaMexico CityMexico

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