Chéour M, Mazlout H, Ben Jalel W, Brour J, Baroudi B, Kraiem A
Service d'Ophtalmologie, Hôpital Habib-Thameur, rue Bab-el-Falah, 2004 Tunis, Tunisie.
J Fr Ophtalmol. 2012 Jan;35(1):46.e1-5. doi: 10.1016/j.jfo.2011.01.022. Epub 2011 Jun 17.
Kindler syndrome is a rare autosomal recessive genodermatosis belonging to the class of bullous poikiloderma. Corneal lesions are rare. We report a case of ocular lesions in this syndrome.
We report the case of a 57-year-old patient followed since childhood in dermatology with the diagnosis of Kindler syndrome. He presented to the ophthalmology department with decreased vision. Ophthalmologic examination showed symblepharon, ectropion in both eyes, and corneal deformation.
The role played by the abnormal protein in epithelial integrity suggests that ocular and more particularly corneal involvement is not rare in Kindler syndrome. In fact, it is less known by ophthalmologists and dermatologists are not aware of the ophthalmologic manifestations.
Kindler综合征是一种罕见的常染色体隐性遗传性皮肤病，属于大疱性皮肤异色症类别。角膜病变较为罕见。我们报告了该综合征的一例眼部病变病例。
我们报告了一名57岁患者的病例，该患者自儿童时期起就因Kindler综合征的诊断在皮肤科就诊。他因视力下降到眼科就诊。眼科检查显示双眼睑球粘连、睑外翻和角膜变形。
异常蛋白在上皮完整性中所起的作用表明，Kindler综合征中眼部尤其是角膜受累并不罕见。事实上，眼科医生对此了解较少，皮肤科医生也未意识到其眼部表现。
