[Rare hyperimmunoglobulinemia E syndromes].

Total immunoglobulin E levels are very frequently measured, particularly for allergy. Their high levels are ordinarily found in atopy and parasitosis. This article subject is to determine rare diseases where high levels of total immunoglobulin E are found except in classic diseases. These rare hyper-immunoglobulin E syndromes are more often associated with immunologic deficiency more or less intense that lead to viral, fungal and bacterial infections. Buckley syndrome: deep infections due to Staphylococcus aureus, chronic dermatitis. Netherton disease: cutaneous and hair disease, allergic manifestations. Wiskott-Aldrich syndrome: thrombocytopeny and -pathy, dermatitis. Di-George disease: hypoparathyroidism, infections. Selective deficit of immunoglobulin A: recurrent infections. It is difficult to differentiate between atopic dermatitis and Buckley syndrome and Netherton disease because of eczematiform chronic dermatitis and high-levels of total immunoglobulin E found in all these diseases.