Brain Abscess and Keratoacanthoma Suggestive of Hyper IgE Syndrome

Brain Abscess and Keratoacanthoma Suggestive of Hyper IgE Syndrome

Hyper immunoglobulin-E (IgE) syndrome is an autosomal immune deficiency disease. It is characterized by an increase in IgE and eosinophil count with both T-cell and B-cell malfunction. Here, we report an 8-year-old boy whose disease started with an unusual skin manifestation. When 6 months old he developed generalized red, nontender nodules and pathologic report of the skin lesion was unremarka...

Renal abscess in hyper-IgE syndrome.

Kidney disease due to Aspergillus fumigatus is a rare finding in hyper-IgE syndrome. We report a patient with autosomal dominant hyper-IgE syndrome, recurrent pneumonia, and acute necrosuppurative pyelonephritis caused by Aspergillus fumigatus with a fatal outcome. We emphasize the severity and the difficulties in the management of renal complications that could be limiting the survival of thes...

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...

Hyper-IgE syndrome

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...