一名儿童的线状回旋状鱼鳞病。对窄谱中波紫外线治疗的反应。
Ichthyosis linearis circumflexa in a child. Response to narrowband UVB therapy.
作者信息
Singer Ralfi, Çopur Mehmet, Yüksel Ece Nur, Kocatürk Emek, Erhan Selma Şengiz
机构信息
Department of Dermatology, Okmeydani Training and Research Hospital, Istanbul, Turkey;
Department of Pathology, Okmeydani Training and Research Hospital, Istanbul, Turkey.
出版信息
J Dermatol Case Rep. 2015 Dec 31;9(4):110-2. doi: 10.3315/jdcr.2015.1214.
BACKGROUND
Ichthyosis linearis circumflexa is a rare form of ichthyosis characterized by polycyclic and annular lesions which are bordered by a double-edged scale. Netherton syndrome is a genodermatosis in which ichthyosis linearis circumflexa is accompanied by characteristic hair shaft abnormalities and atopic diathesis. Different treatment modalities such as emmolients, keratolytics, calcipotriene, topical corticosteroids, topical calcineurin inhibitors, systemic retinoids, phototherapy and photochemotherapy have been used with variable results. Topical therapies may result in considerable absorption because of the defective barrier function in ichthyosis linearis circumflexa/Netherton syndrome.
MAIN OBSERVATION
A 12-year-old patient diagnosed as ichthyosis linearis circumflexa demonstrated considerable improvement with narrowband UVB phototherapy at the end of 30 sessions.
CONCLUSION
Short-term narrowband-UVB may an effective treatment option in patients with ichthyosis linearis circumflexa/Netherton syndrome.
背景
回旋线状鱼鳞病是一种罕见的鱼鳞病，其特征为多环状和环形皮损，边缘有双边鳞屑。Netherton综合征是一种遗传性皮肤病，其中回旋线状鱼鳞病伴有特征性的毛干异常和特应性素质。已使用多种治疗方式，如润肤剂、角质剥脱剂、卡泊三烯、外用糖皮质激素、外用钙调神经磷酸酶抑制剂、系统性维甲酸、光疗和光化学疗法，但效果各异。由于回旋线状鱼鳞病/Netherton综合征的屏障功能缺陷，局部治疗可能会导致大量吸收。
主要观察结果
一名被诊断为回旋线状鱼鳞病的12岁患者在30次窄谱中波紫外线光疗结束时病情有显著改善。
结论
短期窄谱中波紫外线可能是治疗回旋线状鱼鳞病/Netherton综合征患者的有效选择。
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