文献简介

出版社:Clinical and Experimental Dermatology

作  者:T. I. Kaya, U. Tursen and G. Ikizoglu

编  号:10.1111/j.1365-2230.2009.03301.x

关键字:incontinentia pigmenti,incontinentia pigmenti

年  份:2009   点击量:803

文献摘要


Incontinentia pigmenti (IP) is a rare genodermatosis caused by a mutation of nuclear factor kappa B essential modulator gene. There is no specific treatment for IP, therefore it has been claimed that there is no effective treatment to hasten resolution of any of the phases of IP. However, he initial vesiculobullous stage of IP is characterized histopathologically by eosinophilic inflammation, which is expected to respond to corticosteroids. An 18-day-old female neonate was seen, with vesicles on her trunk and limbs diagnosed as the vesiculobullous stage of IP. The patient was treated with a double-compound cream containing a potent corticosteroid (difluocortolone valerate 0.1%) and an antiseptic (chlorquinaldol 1%), to be applied to the lesions twice daily. Five days later, resolution of the lesions was almost complete. As chlorquinaldol has no known anti-inflammatory activity, we attribute this improvement to difluocortolone valerate. This case shows that early lesions of IP with eosinophilic inflammation are treatable.

色素失禁症(IP)是由核因子B kappa基本调节基因的突变导致的一种罕见的遗传性皮肤病。由于没有特殊治疗IP的方法,因此一直声称,没有有效的治疗方法以加快解决IP的任何一个阶段。。然而,IP的初始水疱性阶段以嗜酸性粒细胞的炎症反应为组织病理学特征并且采用皮质类固醇进行治疗。在一个18天的新生女婴身上发现,其躯干和四肢有囊泡存在并被诊断为IP的水疱阶段。患者采用了一种复合乳膏包括一种强效的皮质类固醇(0.1difluocortolone戊酸酯)和抗菌剂(1chlorquinaldol)进行治疗,每日两次涂抹于患处。五天后,病变基本恢复,由于已知双氯甲喹啉没有抗炎活性,我们把改善结果归因于difluocortolone戊酸。这个病例表明,IP的早期病变与嗜酸性粒细胞炎症反应是可以治疗的。