Smith D L, Smith J G, Wong S W, deShazo R D
Department of Medicine, University of South Alabama, Mobile.
J Allergy Clin Immunol. 1995 Jan;95(1 Pt 1):116-23. doi: 10.1016/s0091-6749(95)70159-1.
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 hair 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.
Netherton综合征是一种罕见的症状复合体，其特征为IgE水平大幅升高并伴有特应性表现、一种鱼鳞病性皮肤病（回旋状线状鱼鳞病和/或先天性板层状鱼鳞病）以及特征性毛发异常——套叠性脆发。它可能会与特应性皮炎相混淆，但对局部用皮质类固醇治疗无反应。因此，Netherton综合征对过敏症专科医生具有相当重要的意义。在本文中，我们报告了一名此前未报道患者的临床和免疫学评估结果，该患者对12%乳酸铵洗剂治疗及过敏性疾病管理有反应。此外，我们查阅了英文文献，并将我们患者的发现与其他43例已报道患者的发现进行了比较。我们的患者具有其他此前报道的患有该疾病患者的特征。我们得出结论，这种疾病应被纳入其他IgE升高的疾病之中。患者可能会从对其过敏症状的评估和治疗以及用乳酸铵进行局部皮肤治疗中获益。
