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 unremarkable (inflammatory). Then he developed a painless, cold
abscess. At the age of 4 years, he developed a seronegative polyarticular
arthritis. Another skin biopsy was taken which was in favor of
Keratoacanthoma. Laboratory workup for immune deficiency showed high eosinophil count and high level of
immunoglobulin-E, due to some diagnostic criteria (NIH sores: 41 in 9-year-olds), he was suggestive of
hyper IgE syndrome. At the age of 8, the patient developed an
abscess in the left inguinal region. While in hospital, the patient developed generalized tonic colonic convulsion and
fever. Brain computed tomography scan revealed an
abscess in the right frontal lobe. Subsequently magnetic resonance imaging (MRI) of the brain indicated expansion of the existing
abscess to contralateral frontal lobe (left side). After evacuating the
abscesses and administrating intravenous
antibiotic, the patient's condition improved dramatically and
fever stopped.