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Hyper-IgE syndrome and autoimmunity in Mexican children

Identifieur interne : 001D22 ( Main/Exploration ); précédent : 001D21; suivant : 001D23

Hyper-IgE syndrome and autoimmunity in Mexican children

Auteurs : Marco Yamazaki-Nakashimada [Mexique] ; Samuel Zaltzman-Girshevich [Mexique] ; Silvestre Garcia De La Puente [Mexique] ; Beatriz De Leon-Bojorge [Mexique] ; Sara Espinosa-Padilla [Mexique] ; Marimar Saez-De-Ocariz [Mexique] ; Daniel Carrasco-Daza [Mexique] ; Victor Hernandez-Bautista [Mexique] ; Lorenzo Pérez-Fernandez [Mexique] ; Francisco Espinosa-Rosales [Mexique]

Source :

RBID : ISTEX:E4BE8B4C919BB11C12FE2244E7B435CE7FC58112

English descriptors

Abstract

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.

Url:
DOI: 10.1007/s00467-006-0178-3


Affiliations:


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Le document en format XML

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<div type="abstract" xml:lang="en">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.</div>
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