Okan Fusun, Yapici Zuhal, Bulbul Ali
Department of Neonatology, Sisli Children's Hospital, Halaskargazi cad. Sisli, 34394, Istanbul, Turkey.
Childs Nerv Syst. 2008 Jan;24(1):149-51. doi: 10.1007/s00381-007-0406-6. Epub 2007 Jul 14.
Incontinentia pigmenti is a rare, X-linked dominant multisystem genodermatosis that presents at or soon after birth with characteristic cutaneous signs. The main features occur in the skin where a blistering rash occurs in the newborn period, followed by the blisters becoming raised-like warts. After the skin, the central nervous system is the next most affected system.
We report a female infant who was born with vesicular eruptions who was initially thought to have congenital herpes simplex virus infection.
This case report emphasises that incontinentia pigmenti should be included in the differential diagnosis of cutaneous blistering lesions and central nervous system involvement in neonates.
色素失禁症是一种罕见的X连锁显性多系统遗传性皮肤病,出生时或出生后不久即出现特征性皮肤体征。主要特征出现在皮肤,新生儿期会出现水疱性皮疹,随后水疱会变得像疣一样凸起。除皮肤外,中枢神经系统是下一个受影响最严重的系统。
我们报告一名出生时伴有水疱性皮疹的女婴,最初被认为患有先天性单纯疱疹病毒感染。
本病例报告强调,色素失禁症应纳入新生儿皮肤水疱性病变和中枢神经系统受累的鉴别诊断中。