Patient testimony: hyper-IgE syndrome and associated lung infections

معرفی یک مورد سندرم ازدیاد Hyper-IgE Syndrome) IgE)

Hyper IgE syndrome (Job’s syndrome) is a primary immunodeficiency disease with recurrent infections especially staphylococcal, coarse face, skeletal abnormality and significant increase in serum IgE level (IgE >2000IU/ml). We present a 16 years old boy admitted with chronic cough, dyspnea, eczema and pneumatocele. He had a history of chronic dermal infection since 1 month after birth. The dia...

Recurrent Skin and Lung Infections in Autosomal Dominant Hyper IgE Syndrome with Transactivation Domain STAT3 Mutation

Background. Hyper IgE is a rare systemic disease characterized by the clinical triad of high serum levels of IgE (>2000 IU/mL), eczema, and recurrent staphylococcal skin and lung infections. The presentation of hyper IgE syndrome is highly variable, which makes it easy to confuse the diagnosis with that of severe atopy or other rare immunodeficiency disorders. Case Report. A 23-year-old Hispani...

Hyper-IgE syndrome

Cutis verticis gyrata in a patient with hyper-IgE syndrome.

© 2009 The Authors. doi: 10.2340/00015555-0652 Journal Compilation © 2009 Acta Dermato-Venereologica. ISSN 0001-5555 Sir, Cutis verticis gyrata (CVG) is a rare morphological condition of the scalp characterized by ridges and furrows resembling the surface of the brain. We report here a case of CVG in a patient with hyper-IgE syndrome, which is a multisystem disorder known to affect the dentitio...

Autosomal dominant hyper-IgE syndrome

Th e hyper-IgE recurrent infection syndromes (HIES) comprise a group of primary immunodefi ciency dis orders that exhibit markedly elevated IgE levels, recur rent staphylococcal skin abscesses, eczema and pulmonary infections. Both autosomal dominant and autosomal recessive forms of the disorder have been described. Most autosomal dominant HIES (AD-HIES) have been found to be due to mutations i...