NETHERTONS SYNDROME - A SYNDROME OF ELEVATED IGE AND CHARACTERISTIC SKIN AND HAIR FINDINGS

Netherton's syndrome is a rare symptom complex characterized by greatly elevated IgE levels with atopic manifestations, an ichthyotic skin disorder (ichthyosis linearis circumflexa and/or congenital lamellar ichthyosis), and the characteristic hail abnormality trichorrhexis invaginata. It may be confused with atopic dermatitis but does not respond to topical corticosteroid treatment Netherton's syndrome is therefore of considerable importance to allergists. In this article we report the results of the clinical and immunologic evaluation of a previously unreported patient who responded to treatment with 12% ammonium lactate lotion and management of his allergic disease. Furthermore, we have reviewed the English literature and compared the findings in our patient with those of 43 other reported patients. Our patient shared the features of other previously reported patients with this disorder. We conclude that this disease should be included among the other disorders with elevated IgE. Patients may benefit from evaluation and treatment of their allergic symptoms and topical skin treatment with ammonium lactate.