BACKGROUND Hyper IgE syndrome (HIES) is a rare primary immune deficiency, described as Job(`)s syndrome characterized by increased serum levels of IgE, eczema, recurrent cutaneous and pulmonary infections. In this paper, we presented a case of Hyper IgE syndrome. CASE PRESENTATION A 16-year-old Iranian boy presented with a one year history of skin lesions in knees and elbows was diagnosed of ...

BACKGROUND Atopic eczema is a common childhood disease associated with high IgE and eosinophilia. We characterized the clinical features associated with hyper-IgE (defined as IgE > 2000 IU/L) in eczema. METHODS Nottingham Eczema Severity Score (NESS), family and personal history of atopy, skin prick test (SPT) for common food and aeroallergens, highest serum IgE ever and eosinophil counts wer...

Kimura’s disease (KD) is a chronic inflammatory disorder primarily seen in male Asians during the second and third decades of life. Clinically, it presents as solitary or multiple subcutaneous nodules, predominantly in the head and neck region, typically in the pre auricular region, forehead, and scalp. The etiology of Kimura disease is still unknown. This disorder should be suspected when the ...

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-immunoglobulin E (IgE) is a disorder of immune system characterized by assemblage symptoms including recurrent skin and pulmonary infections, skeletal anomalies elevated serum IgE (>2000 IU/ml). More recently, apart from the typical features, neurological manifestations have also been described central nervous facial nerve palsy demyelinating disorders. Here we describe three patients ...

Dubowitz syndrome is a rare, autosomal recessive disorder characterized by intrauterine growth retardation, short stature, microcephaly, distinct facial dysmorphism, and psychomotoric retardation. The hyper-immunoglobulin E (hyper-IgE or Job syndrome) is a primary immunodeficiency characterized by recurrent staphylococcal abscesses, recurrent cyst-forming pneumonia, and an elevated serum IgE le...