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01.08.2006 | Brief Report

Hyper-IgE syndrome and autoimmunity in Mexican children

verfasst von: Marco Yamazaki-Nakashimada, 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

Erschienen in: Pediatric Nephrology | Ausgabe 8/2006

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Abstract

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.

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Titel: Hyper-IgE syndrome and autoimmunity in Mexican children
verfasst von: Marco Yamazaki-Nakashimada
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
Publikationsdatum: 01.08.2006
Verlag: Springer Berlin Heidelberg
Erschienen in: Pediatric Nephrology / Ausgabe 8/2006
Print ISSN: 0931-041X
Elektronische ISSN: 1432-198X
DOI: https://doi.org/10.1007/s00467-006-0178-3

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