HYPER-IGE SYNDROME: A CASE DESCRIPTION

The spectrum of hyper-IgE syndrome include severe atopic dermatitis lesions, elevated serum IgE-levels, repeated occurrence of skin and respiratory tract infections, dental and skeletal abnormalities. It is a rare genetic multiorgan disorder with an incidence of one in 500,000 and onset in early childhood. The more frequent modality of inheritance is as a single-locus autosomal dominant trait with variable expression. The differential diagnosis include Wiscott-Aldrich syndrome and Langerhans cell histiocytosis. We report on a 6-year-old boy, who disclosed the characteristic face, atopic dermatitis lesions with chronically relapsing course since the first month of life, delayed eruption of permanent teeth, recurrent skin, gastroenteric and respiratory tract infections. Upon investigations his serum IgE levels were grossly elevated (4117.9 U/ml; normal range: 1.3–165.3), eosinophils 13%; Haemoglobin 10.6 mg/L; inflammatory parameters were all elevated. Culture from skin swabs grew Proteus mirabilis; pharyngeal and nasal swabs grew Staphylococcus aureus. Treatment with monohydrate cephaclor 250 mg daily stopped the skin and respiratory tract infections, antihistaminic reduced the severe itch, while medications with medium potency topical steroids and fusidic acid cream ameliorated the eczematous lesions. Beside, relapses were noted every time the systemic treatments were discontinued. Long-term chemoprophylaxis is a major problem in this disease.