Department of Dermatology, School of Medicine, Mersin University, Mersin, Turkey.
Clin Exp Dermatol. 2009 Dec;34(8):e611-3. doi: 10.1111/j.1365-2230.2009.03301.x. Epub 2009 Jun 1.
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, the 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.
遗传性皮肤病（IP）是一种罕见的基因皮肤病，由核因子 kappa B 必需调节剂基因突变引起。目前尚无针对 IP 的特定治疗方法，因此有人声称，没有有效的治疗方法可以加速 IP 任何阶段的消退。然而，IP 的初始水疱疱状阶段在组织病理学上表现为嗜酸性炎症，预计对此类炎症对皮质类固醇有反应。我们接诊了一名 18 天大的女婴，她的躯干和四肢有水疱，被诊断为 IP 的水疱疱状阶段。患者接受了一种含有强效皮质类固醇（双氟可龙戊酸 0.1%）和防腐剂（氯喹那多 1%）的复方乳膏治疗，每天两次涂抹于皮损处。五天后，皮损几乎完全消退。由于氯喹那多没有已知的抗炎活性，我们认为这种改善是由于双氟可龙戊酸所致。这个病例表明，具有嗜酸性炎症的 IP 早期皮损是可以治疗的。
