眼瘢痕性类天疱疮

瘢痕性类天疱疮是一种少见的慢性、进行性自身免疫性大疱性疾病,主要累及黏膜,以瘢痕化为特征。眼部受累时结膜炎症、溃疡、瘢痕形成,继发角膜受损、混浊而视力下降。仅有眼部受累时诊断较为困难,如治疗不及时,有25％的患者视力丧失。本文就眼瘢痕性类天疱疮的定义、病因及发病机制、眼部表现、分期、诊断、鉴别诊断、治疗等进行综述。     

甲氨蝶呤治疗眼瘢痕性类天疱疮的疗效

目的：探讨应用甲氨蝶呤（MTX）治疗眼瘢痕性类天疱疮（OCP）的效果及安全性。方法：回顾性系列病例研究。收集2016年9月至2019年8月于天津医科大学总医院眼科行MTX治疗的OCP患者5例（10眼）,其中男2例,女3例。观察MTX治疗6个月、12个月OCP炎症的进展及药物不良反应。结果：本组患者OCP平均发病年龄51.8岁（38～66岁）,开始免疫治疗年龄54.4岁（39～68岁）。MTX治疗6个月,OCP好转3例,OCP部分活跃1例,OCP活跃1例。MTX治疗12个月,OCP好转4例,OCP活跃1例。活跃患者OCP睑球粘连进展,在MTX治疗12个月后加用利妥昔单抗联合治疗。治疗过程中,1人（1眼）在MTX治疗10个月时发生角膜病毒感染。另有2例应用MTX后出现轻度胃肠道反应,1例转氨酶轻度升高。随访期间无其他严重不良反应出现。结论：对于OCP患者,MTX耐受良好,早期应用有助于控制眼部病情。     

眼瘢痕性类天疱疮的治疗策略

眼瘢痕性类天疱疮（ OCP）是一种罕见的累及眼结膜的自身免疫病,常由于其早期的非特异性临床表现导致误诊或延误治疗,最终造成眼表结构功能损害及失明。 OCP治疗主要通过全身免疫抑制控制炎症反应及瘢痕进展,经过多年的临床探索及众多新药问世,目前治疗期前景乐观。现全身免疫抑制治疗主要包括传统免疫抑制治疗（CIST）及新型免疫抑制治疗。 CIST现主要为治疗OCP的一线用药,但由于其副作用较多、对顽固性OCP疗效不佳而有一定缺陷;新型免疫抑制药物如IVIg、抗TNF-α药及单克隆抗体等,凭借强力的免疫抑制作用及较少副反应逐渐受到重视,在今后可能可作为一线用药的更优选择。中晚期OCP患者因并发眼表畸形及角膜浑浊等严重影响生活质量,在全身炎症情况控制稳定的前提下可行手术治疗。局部治疗主要针对不同的眼表并发症行相应处理,以改善局部症状。经过科学合理的药物及手术治疗,大部分OCP患者预后良好,但仍有少量反复发作者需进一步研究,寻找最为合适的治疗策略。     

眼型瘢痕性类天疱疮一例

眼型瘢痕忡类天疱疮（ocular eicatricial pemphigoid.OCP）临床上较少见．预后较差现将我院收治的1例报告如下.     

应用免疫荧光技术对眼瘢痕性类天疱疮的研究

目的 探讨免疫荧光技术对眼瘢痕性类天孢疮（ＯＣＰ）的实验诊断意义。方法 应用直接免疫荧光（ＤＩＦ）检测１６例ＯＣＰ患者的结膜和应用间接免疫荧光（ＨＦ）以鼠食道和正常人结膜为基质检测３４例ＯＣＰ患者的血清。结果 ＤＩＦ证实６例结膜上皮细胞基底膜上有免疫复合物（ＩｇＧ和／或ＩｇＡ）和／或Ｃ３呈线状沉积。以结膜为基质的ＨＦ证实血清中抗基底膜（ＢＭＺ）抗体４例,抗细胞间物质（ＨＳ）抗体１４例,总阳性率为５     

瘢痕性类天疱疮角膜病变手术治疗

目的 观察眼瘢痕性类天疱疮(OCP)行羊膜移植和穿透性角膜移植术(PKP)后的效果.设计 回顾性病例系列.研究对象 OCP患者3例(4眼).方法 2例(3眼)患者为三级睑球粘连,术前视力HM/5cm～HM/10cm,行睑球粘连分离联合羊膜移植,术后放置绷带性角膜接触镜;另外1例(1眼)因并发角膜溃疡伴穿孔,行PKP,供体为甘油冷冻保存的角膜,观察术后角膜、结膜愈合情况.主要指标 视力,角膜、结膜愈合情况.结果 羊膜移植2例(3眼)患者术后1个月羊膜逐步吸收,2例(2眼)手术1年后视力0.04～0.05,中央角膜上皮化良好,二级睑球粘连,角膜缘新生血管减少.PKP后3周植片融解严重,1个月后行全角膜移植联合眼前节切除,再次发生植片融解,眼内容物流失而眼球萎缩.结论 部分眼表功能不全的OCP患者睑球粘连分离联合羊膜移植对减轻睑球粘连、提高视力有一定意义;由于多种不利因素影响,穿透性角膜移植治疗OCP并发穿孔性角膜溃疡效果不良.     

瘢痕性类天疱疮眼部病变一例

瘢痕性类天疱疮（cicatricial pemphigoid,CP)是一种少见的系统性自身免疫性疾病，其特征是皮肤黏膜疱性溃疡和瘢痕形成，眼部的主要表现是慢性角结膜炎，伴有结膜上皮下瘢痕形成，导致穹隆部缩短、睑球粘连、睑缘粘连，睑内翻，倒睫，干眼症、角膜新生血管和瘢痕形成，预后较差。我院于2001年诊治1例CP患者，现报告如下。     

眼瘢痕性类天疱疮病例的回顾性分析

目的 探讨眼瘢痕性类天疱疮(OCP)的临床特点.方法 回顾性系列病例研究.回顾2005年1月至2008年10月于北京大学第一医院眼科和皮肤科联合确诊为OCP的系列病例5例(10只眼)患者的病史、眼科常规检查、结膜囊细菌培养及全身免疫学常规检查的结果,分析其误诊原因.结果 确诊前,5例患者在早期均误诊为慢性结膜炎,在这些病例瘢痕较明显时,1例曾被误诊为Stevens-Johnson综合征,1例误诊为干燥综合征,患者从首次就诊至确诊的时间长达2～5年.在确诊前均经过长期抗菌滴眼液的局部治疗,1例经3次内翻倒睫矫正术,术后病情加重.5例患者中诊断为OCPⅡ期3只眼、Ⅲ期5只眼、Ⅳ期2只眼,3例患者结膜囊细菌培养阳性,仅1例患者肿瘤标记物铁蛋白轻度升高,其余患者常规免疫学检查均正常.确诊后经合理治疗病情控制,但2例患者结膜瘢痕继续进展.结论 OCP早期表现类似慢性结膜炎,提高对OCP的警惕和认识是避免误诊的重要手段.     

眼瘢痕性类天疱疮10年经历诊治一例

患者女,71岁。2004年2月因“双眼干涩、疼痛、异物感”,先后至多家医院就诊,均诊断为“结膜炎”或“角膜炎”,给予双眼局部药物治疗（具体不详）,症状未见好转。2011年1月患者至北京同仁医院就诊,明确诊断为“双眼瘢痕性类天疱疮”,给予环孢素眼液点眼治疗1年余,患者自觉治疗好转,但双眼视力逐渐下降,2012年5月就诊于北大人民医院.     

Ocular cicatricial pemphigoid

Ocular cicatricial pemphigoid is a chronic progressive immunological disease, although acute inflammatory episodes may occur. Typically, it leads to fornix foreshortening, symblepharon and dry eye problems. In the end stage, the cornea is severely affected, resulting in serious vision loss. We report on the diagnosis and management of a patient with ocular cicatricial pemphigoid and comment on the differential diagnosis, complications and treatment options.     

Ocular cicatricial pemphigoid

A series of 17 patients with ocular cicatricial pemphigoid (OCP) is described retrospectively. The importance of early recognition, especially of disease involving the medial canthus and caruncular region, diagnosis by biopsy, and adequate immunosuppressive and surgical therapy, are emphasised.
A detailed grading scheme has been developed and this has enabled the authors to determine the success or failure of the therapy during the active treatment period. A combined ophthalmological and immunological approach to treatment can result in a successful visual outcome of this potentially blinding disease.     

Eosinophil granule proteins expressed in ocular cicatricial pemphigoid

BACKGROUND—Blister formation and tissue damage in bullous pemphigoid have been attributed to the release of eosinophil granule proteins—namely, to eosinophil derived cationic protein (ECP) and major basic protein (MBP). In the present investigation these eosinophil granule proteins were studied in the conjunctiva of patients with ocular cicatricial pemphigoid (OCP).
METHODS—Conjunctival biopsy specimens obtained from patients with subacute (n=8) or chronic conjunctival disease (n=13) were analysed histologically and immunohistochemically using antibodies directed against EG1 (stored and secreted ECP), EG2 (secreted ECP), MBP, CD45 (common leucocyte antigen), CD3 (pan T cell marker), and HLA-DR (class II antigen).
RESULTS—Subepithelial mononuclear cells, mast cells, and neutrophils were detected in all specimens. The number of mononuclear cells, neutrophils, CD45+ cells, CD3+ cells, and the HLA-DR expression were significantly higher in the subacute than in the chronic disease group. Some eosinophils were found in specimens from five of eight patients with subacute OCP, but in none of the patients with chronic disease. The eosinophil granule proteins (ECP and MBP) were found in the epithelium and substantia propria in patients with subacute conjunctivitis.
CONCLUSIONS—Subepithelial cell infiltration in the conjunctiva greatly differs between subacute and chronic ocular cicatricial pemphigoid specimens. The findings suggest that eosinophil granule proteins may participate in tissue damage in acute phase of inflammation in OCP.

     

Metotrexato como tratamiento en el penfigoide ocular cicatricial moderado

Clinical caseA 73 year-old woman presented with a history of non-specific symptoms and photophobia in both eyes of 1 year progression. The examination revealed a bilateral symblepharon and fornix shortening. Immunohistochemical analysis confirmed the presence of linear deposits of IgG, IgM and C3 along the conjunctival basement membrane. With the diagnosis of Ocular Cicatricial Pemphigoid, systemic treatment with subcutaneous methotrexate was prescribed.DiscussionWe consider such treatment a very effective initial immunosuppressive alternative in patients with moderate conjunctival inflammation and in cases of rapid progression.     

Conjunctival mucoepidermoid carcinoma in a patient with ocular cicatricial pemphigoid and a review of the literature

Invasive mucoepidermoid carcinoma of the conjunctiva of the left lower eyelid was diagnosed in an orbital exenteration specimen of a 57-year-old woman, after a biopsy of the same lesion was originally diagnosed as invasive squamous cell carcinoma. The woman was undergoing mitomycin C injections for ocular cicatricial pemphigoid, diagnosed in the same eye 2 years prior to identification of the neoplasm. The tumor invaded the cornea, sclera, lacrimal gland, regional small nerves, and lymphatics, but did not show intraocular involvement. The original biopsy specimen was reassessed with stains for mucin and found to be mucoepidermoid carcinoma of the conjunctiva. We reviewed 21 cases of mucoepidermoid carcinoma of the conjunctiva described to date in the English literature. We believe this number underestimates the true incidence of this condition, as it is frequently misdiagnosed both clinically and histopathologically. Evaluating suspected aggressive squamous cell carcinoma with special stains for mucin generally helps to identify mucoepidermoid carcinoma of the conjunctiva. More extensive surgical excision than that used for squamous cell carcinoma should be implemented in the management of mucoepidermoid carcinoma of the conjunctiva to prevent recurrence.     

Incidence, prevalence, and demographic characteristics of ocular cicatricial pemphigoid in Colombia: data from the National Health Registry 2009-2019

AIM: To provide an epidemiological characterization of ocular cicatricial pemphigoid (OCP) in Colombia. METHODS: We conducted a cross-sectional study using SISPRO. We applied the specific code of the International Classification of Diseases for Ocular Pemphigoid, from 2009-2019 to estimate prevalence, incidence, and the demographic status of the disease in Colombia. RESULTS: The estimated average prevalence was 0.22 per 1 000 000 inhabitants, and the estimated average incidence was 0.24 per 1 000 000 inhabitants. With a female predominance of 62.5%, and a male/female ratio of 1:1.6. The group of patients diagnosed with the disease after the age of 80 presented the highest prevalence. The departments with the highest prevalence were Antioquia, Bogotá, and Santander. CONCLUSION: There are important differences between worldwide and Colombian prevalence and incidence data, which may be related to genetic and epigenetic factors, and the possible underdiagnosis of the disease. According to the results, OCP is an extremely rare disease in Colombia. Nevertheless, it is important to encourage awareness of the disease due to its devastating consequences.     

Long term results of systemic chemotherapy for ocular cicatricial pemphigoid

Ocular cicatricial pemphigoid (OCP) is a chronic, progressive, blinding, autoimmune disease that scars mucous membranes. We studied the long-term outcome in 104 consecutive patients (average follow-up: 4 years) to determine whether complete remission could be achieved following a course of treatment with immunosuppressive drugs. We found that prolonged periods of remission off therapy are maintained in about one third of OCP patients. Follow-up must be continued for life as relapse occurs in approximately one third of cases. Those who relapsed regained disease control readily upon reinstitution of therapy and did not deteriorate to more advanced cicatrization. Sex, age, initial degree of inflammation and the incidence of extraocular involvement did not bear a prognostic significance. The mechanism which underlies the differing responses to therapy is not yet known.Abbreviations BMZ basement membrane zone - CP cicatricial pemphigoid - OCP ocular cicatricial pemphigoid - OD right eye - OS left eye