[Case presentation of a patient with hyper immunoglobulin E syndrome]

ABSTRACT

IgE syndrome, also known as Job's syndrome, is a very rare cause of immunodeficiency. It is presented with recurrent staphylococcal abscesses in the skin, lungs, and other organs as well as itching dermatitis since the early stages of life. Significant increase serum level of IgE, eosinophilia, and normal serum levels of other immunoglobulines are the major features of this abnormality. Our patient was a 12 month old girl with presenting symptoms of itching dermatitis and recurrent skin and lung infections. She also had the history of umbilical chord infection as well as several attacks of dysentery. On physical examination she was pale and had a coarse face. She also had symptoms of mild malnutrition and several erythematous papules covered her body. Serum level of IgE was more than 150 IU/ml, while serum levels of the other immunoglobulines and complement were within normal limits. These findings established the diagnosis of hyper IgE syndrome for the patient. Combination of itching dermatitis, recurrent skin and lung infections, and nail dystrophy as well as significant increase in serum IgE level, suggests hyper IgE syndrome as the most likely diagnosis