title: Hyper-IgE syndrome and autoimmunity in Mexican children.
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.
authors.affiliation: Department of Clinical Immunology, Instituto Nacional de Pediatría, Insurgentes Sur 3700-C, Col. Insurgentes Cuicuilco, Mexico City, Mexico. yzki71@yahoo.com.mx
authors.forename: Marco
authors.initials: M
authors.lastname: Yamazaki-Nakashimada
authors.forename: Samuel
authors.initials: S
authors.lastname: Zaltzman-Girshevich
authors.forename: Silvestre
authors.initials: S
authors.lastname: Garcia de la Puente
authors.forename: Beatriz
authors.initials: B
authors.lastname: De Leon-Bojorge
authors.forename: Sara
authors.initials: S
authors.lastname: Espinosa-Padilla
authors.forename: Marimar
authors.initials: M
authors.lastname: Saez-de-Ocariz
authors.forename: Daniel
authors.initials: D
authors.lastname: Carrasco-Daza
authors.forename: Victor
authors.initials: V
authors.lastname: Hernandez-Bautista
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Pérez-Fernandez
authors.forename: Francisco
authors.initials: F
authors.lastname: Espinosa-Rosales
mesh_terms: D001327:Autoimmune Diseases / Q000276:immunology*; D015551:Autoimmunity; D002648:Child; D005260:Female; D006801:Humans; D007589:Job Syndrome / Q000150:complications* / Q000276:immunology*; D008297:Male; D008800:Mexico