【摘要】 目的：探讨瘢痕性天疱疮临床特点，为临床诊治提供帮助。方法：回顾性分析200201/200801我科诊治的35例瘢痕性天疱疮临床特点。结果：瘢痕性天疱疮平均发病年龄为62.4岁，男女性别比为20∶15，病史平均3.3a，51.43%以口腔黏膜病变首发，31.43%以眼部病变首发，眼睑内翻倒睫/慢性结膜炎和干眼/角膜病变为5/4/2，17.14%以其它黏膜病变首发。经过治疗，71.43%(25/35)患者症状消失或缓解超过3mo，14.29%(5/35)患者症状反复或无明显缓解，14.29%(5/35)患者中途退出治疗。治疗过程中37.14%(13/35)患者出现全身其它异常，其中6例出现肝功能异常，2例终止治疗，肾功能异常2例，1例终止治疗，血液系统异常3例，1例退出治疗，心肌梗塞1例，退出治疗，明显血压升高1例。结论：瘢痕性天疱疮是一种慢性病，多发于老年人，男性稍多于女性，眼组织经常受累，多表现为干眼症、睑球粘连和内翻倒睫。瘢痕性天疱疮治疗复杂，经常需要多学科协作。
【关键词】 瘢痕性天疱疮;睑球粘连;干眼症
Analysis of clinical characteristics of cicatricial pemphigoid in 35 cases
XiaoYing Hui 1, HongBing Zhang2
1Department of Ophthalmology，Fuping Chinese Medicine Hospital, Fuping County 711700, Shaanxi Province,China;2 Shaanxi Institute of Ophthalmology, Xian Eye Hospital，the First Hospital of Xian,Xian 710002, Shaanxi Province,China
Correspondence to:HongBing Zhang.Shaanxi Institute of Ophthalmology, Xian Eye Hospital，the First Hospital of Xian,Xian 710002, Shaanxi Province,[email protected]
Received:20100521 Accepted:20100713
Abstract
AIM: To explore the clinical characteristics of ocular cicatricial pemphigoid, and provide assistance in clinical diagnosis and therapy.
METHODS: The data of 35 cases of ocular cicatricial pemphcgoid from January, 2002 to January, 2008 was retrospectively analyzed.
RESULTS:The average age of ocular cicatricial pemphigoid was 62.4 years, and the rate of male and female gender was 20/15. The average history was 3.3 years. The oral mucosa was firstly invaded in 51.43% patients, ocular mucosa in 31.43% ones, and other mucosa in 17.14% ones. In eyeinvolved patients, the rate of eyelid entropium and trichiasis, dry eye and chronic conjunctivitis, and corneopathy was 5/4/2. After treatment, 71.43%(25/35) cases were cured, while 14.29% (5/35)cases were not cured, and 14.29%(5/35)cases did not accomplished the regimen. In the course of treatment, the complications were found in 37.14% (13/35)cases, and among these, abnormal liver function was found in 6 cases and 2 cases withdrew from treatment, abnormal kidney function in 2 cases and 1 case discontinued the protocal, abnormal blood system in 3 cases and 1 case quitted the regimen, myocardial infarction in 1 case who gave up the treatment, and apparent blood hypertension in 1 case.
CONCLUSION: Cicatricial pemphigoid is a chronic ailment and often involved in the elderly. Male patients are more than female ones, and ocular tissue is often involved. Dry eye disease, symblepharon, entropion and trichisis are all its manifestations. The treatment of cicatricial pemphigoid is complicated, and multidisciplinary collaboration is required.
KEYWORDS: cicatricial pemphigoid; symblepharon; dry eye disease
Hui XY, Zhang HB. Analysis of clinical characteristics of cicatricial pemphigoid in 35 cases. Int J Ophthalmol (Guoji Yanke Zazhi) 2010;10(8):18291831
瘢痕性天疱疮是一种慢性自身免疫性疾病，多发生于老年人皮肤和黏膜组织，这种疾病国外基础研究和临床报道很多[19]，国内相对较少[1012]，最近我们在临床工作中发现这种疾病在我们国家并不少见，却常常诊断为慢性结膜炎和干眼症等眼表疾病，现总结我院200201/200801诊治的35例该病临床资料，以提高眼科医生对该病的认识。
1对象和方法
1.1对象
患者35例,男20例，女15例，年龄45～74(平均62.4)岁;随访时间6mo～7a，平均3.3a。以口腔黏膜病变为首发症状者18例，以眼部症状为首发者11例，其中以眼睑内翻倒睫为首发症状者5例，慢性结膜炎和干眼为首发症状者4例，以角膜病变视力受损为首发症状者2例，而以呼吸道、食道和/或泌尿生殖道黏膜病变为首发症状者6例。
1.2方法
诊断标准：(1)具有眼部、口腔、呼吸道和/或泌尿生殖道不适的症状;(2)眼部不明原因结膜慢性充血、进展性结膜收缩、穹隆缩短，甚至干眼症、睑球粘连、睑内翻倒睫和角膜混浊;(3)黏膜病检发现黏膜下大量炎性细胞浸润和/或瘢痕形成(图1，2);(4)可伴有身体其他部位皮肤或黏膜损害，如口腔黏膜水泡或大疱，以及瘢痕;皮肤红斑和/或萎缩性瘢痕;(5)排除口腔黏膜干燥综合征和大泡性天疱疮等黏膜干燥性疾病。对确诊患者，治疗开始前向患者和家属详细介绍该病发生原因和治疗现状，取得患者和家属支持和同意后签署知情同意书，开始治疗。治疗方法：(1)以干眼为首发症状者眼部常规应用人工泪液和类固醇激素眼药水，效果不明显时加用免疫抑制剂眼药水;(2)明显眼睑内翻倒睫和睑球粘连者，在炎症完全稳定3mo后，行内翻倒睫矫正、睑球粘连分离、结膜囊成型和羊膜移植手术;(3)角膜病变严重且视力<0.1者，眼部炎症控制、病情稳定3mo者行穿透性角膜移植手术，角膜新生血管明显者联合角膜缘干细胞移植，视力>0.1者保守治疗;(4)以呼吸系统、消化系统和/或生殖系统受损为首发症状者首诊于相关科室，确诊或疑诊但是合并眼部症状者请眼科会诊，共同指定治疗方案;(5)以眼部不适为首发症状的患者行眼部治疗的同时，请其它相关科室会诊，确定有无其它器官病变;(6)单纯眼部病变者仅行眼部治疗，合并其它器官病变系统治疗，轻度和中度患者全身应用氨苯砜，重度和无效患者全身应用皮质类固醇激素和环磷酰胺;(7)治疗期间定期检查肝脏、肾脏、心血管和血液系统功能，出现异常者请相关科室会诊，及时调整治疗方案，必要时停止治疗;(8)病情稳定1mo者出院;(9)随访1次/mo。
统计学分析：观察结果用SPSS 11.0软件进行率的描述。P<0.05为差异有统计学意义。
2结果
临床治愈标准：(1)停用激素或环磷酰胺，患者不用或仅用人工泪液,眼部无不适症状，全身无其它不适，时间超过3mo。(2)眼部和其它组织器官检查无明显充血等炎症体征。(3)组织病检黏膜下可有瘢痕组织形成，但是无明显炎性细胞浸润。
瘢痕性天疱疮患者35例中，51%(18/35)病例以口腔黏膜病变首发，31%(11/35)以眼部病变首发，眼睑内翻倒睫/慢性结膜炎和干眼/角膜病变为5/4/2，17%(6/35)以其它黏膜病变首发。经过治疗，71.43%(25/35)患者症状消失或缓解超过3mo，14.29%(5/35)患者症状反复或无明显缓解，14.29%(5/35)患者中途退出治疗。治疗过程中37.14%(13/35)患者出现全身其它异常，其中6例出现肝功能异常，2例终止治疗，肾功能异常2例，1例终止治疗，血液系统异常3例，1例退出治疗，心肌梗塞1例，退出治疗，明显血压升高1例。图1大量炎性细胞浸润(HE×40)。图2明显瘢痕组织形成(HE×40)。
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