Hyper IgE (Job’s) syndrome: a primary immune deficiency with oral manifestations

Cutaneous manifestations of hyper-IgE syndrome in infants and children.

We describe 8 children with hyper-IgE syndrome who had papulopustular eruption on the face and scalp in the first year of life. Seven of the 8 patients had persistent peripheral eosinophilia and 3 had leukocytosis noted before diagnosis. Skin biopsy specimens in 6 patients revealed spongiosis and perivascular dermatitis and/or folliculitis with a predominance of eosinophils. Two patients had bo...

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...

Psoriasis in hyper IgE syndrome – a case report

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 pso...

Hyper-IgE syndrome

Clinical Manifestations of Hyper IgE Syndromes

Over the last 4 years, three genetic etiologies of hyper IgE syndromes have been identified: STAT3, DOCK8, and Tyk2. All of these hyper IgE syndromes are characterized by eczema, sinopulmonary infections, and greatly elevated serum IgE. However, each has distinct clinical manifestations. Mutations in STAT3 cause autosomal dominant HIES (Job's syndrome), which is unique in its diversity of conne...