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

Netherton's syndrome is a rare symptom complex characterized by greatl y elevated IgE levels with atopic manifestations, an ichthyotic skin d isorder (ichthyosis linearis circumflexa and/or congenital lamellar ic hthyosis), and the characteristic hail abnormality trichorrhexis invag inata. 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 th e results of the clinical and immunologic evaluation of a previously u nreported patient who responded to treatment with 12% ammonium lactate lotion and management of his allergic disease. Furthermore, we have r eviewed the English literature and compared the findings in our patien t with those of 43 other reported patients. Our patient shared the fea tures of other previously reported patients with this disorder. We con clude that this disease should be included among the other disorders w ith elevated IgE. Patients may benefit from evaluation and treatment o f their allergic symptoms and topical skin treatment with ammonium lac tate.