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Title

HYPERIMMUNOGLOBULIN E SYNDROME: GENETICS, IMMUNOPATHOGENESIS, CLINICAL FINDINGS, AND TREATMENT MODALITIES

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Abstract

 The HYPERIMMUNOGLOBULIN E SYNDROMEs (HIESs) are very rare IMMUNODEFICIENCY syndromes with multisystem involvement, including immune system, skeleton, connective tissue, and dentition. HIES are characterized by the classic triad of high serum levelsof immunoglobulin E (IgE), recurrent staphylococcal cold skin abscess, and recurrent pneumonia with pneumatocele formation.Most cases of HIES are sporadic although can be inherited as autosomal dominant and autosomal recessive traits. A fundamentalimmunologic defect in HIES is not clearly elucidated but abnormal neutrophil chemotaxis due to decreased production or secretionof interferon γ has main role in the immunopathogenesis of syndrome, also distorted Th1/Th2 cytokine profile toward a Th2 biascontributes to the impaired cellular immunity and a specific pattern of infection susceptibility as well as atopic‑allergic constitutionof syndrome. The ophthalmic manifestations of this disorder include conjunctivitis, keratitis, spontaneous corneal perforation, recurrent giant chalazia, extensive xanthelasma, tumors of the EYElid, strabismus, and bilateral keratoconus. The diagnosis of HIESis inconclusive, dependent on the evolution of a constellation of complex multisystemic symptoms and signs which develop overthe years. Until time, no treatment modality is curative for basic defect in HIES, in terms of cytokines/chemokines derangement. Ofnote, bone marrow transplant and a monoclonal anti‑IgE (omalizumab) are hoped to be successful treatment in future.

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