Thorne Jennifer E, Anhalt Grant J, Jabs Douglas A
Department of Ophthalmology, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA.
Ophthalmology. 2004 Jan;111(1):45-52. doi: 10.1016/j.ophtha.2003.03.001.
To describe the clinical characteristics of patients with mucous membrane pemphigoid (MMP) and pseudopemphigoid.
Retrospective cohort study.
Two hundred eighty consecutive patients referred for the evaluation of possible ocular MMP from January 1, 1985, to December 31, 2001.
Information on patients presenting for evaluation of possible MMP was entered prospectively into a database, which was supplemented by a retrospective chart review. Mucous membrane pemphigoid was diagnosed in patients with a compatible clinical picture by the linear deposition of antibodies to the basement membrane zone (BMZ) on direct immunofluorescent analysis of a mucous membrane biopsy specimen or by the presence of circulating autoantibodies to epithelial BMZ.
Demographic and clinical characteristics of MMP and pseudopemphigoid; risk of ocular MMP among patients presenting with extraocular MMP without ocular disease.
Among patients with ocular MMP, extraocular disease was common (82.4% of patients). The risk of ocular involvement among patients with MMP seen without ocular disease was approximately 5% per year over the first 5 years of follow-up (cumulative risk at 5 years, 22%). Although immunohistologic confirmation of the diagnosis was obtained in all patients, the initial conjunctival biopsy was positive for MMP in 80% of the patients diagnosed with ocular MMP. The most frequent presumed causes of pseudopemphigoid were topical glaucoma medications (28.3%), rosacea blepharoconjunctivitis (20.0%), atopic keratoconjunctivitis (8.3%), and conjunctival lichen planus (8.3%).
Patients with ocular MMP typically have other systemic manifestations of MMP. Patients who are initially seen with extraocular MMP without ocular involvement are at risk for ocular disease developing. The clinical characteristics of ocular MMP and pseudopemphigoid are similar; therefore, immunohistologic evaluation of biopsied tissue is needed to confirm the diagnosis of MMP.
描述黏膜类天疱疮（MMP）和类天疱疮样病变患者的临床特征。
回顾性队列研究。
1985年1月1日至2001年12月31日期间连续转诊来评估可能患有眼部MMP的280例患者。
将前来评估可能患有MMP的患者信息前瞻性地录入数据库，并通过回顾性病历审查进行补充。通过对黏膜活检标本进行直接免疫荧光分析，若基底膜带（BMZ）抗体呈线性沉积，或存在针对上皮BMZ的循环自身抗体，则诊断为黏膜类天疱疮。
MMP和类天疱疮样病变的人口统计学和临床特征；无眼部疾病的眼外MMP患者发生眼部MMP的风险。
眼部MMP患者中，眼外疾病很常见（82.4%的患者）。在最初无眼部疾病的MMP患者中，随访的前5年每年眼部受累风险约为5%（5年累积风险为22%）。虽然所有患者均获得了诊断的免疫组织学确认，但在诊断为眼部MMP的患者中，80%的患者最初结膜活检MMP呈阳性。类天疱疮样病变最常见的推测病因是局部青光眼药物（28.3%）、酒渣鼻性睑结膜炎（20.0%）、特应性角结膜炎（8.3%）和结膜扁平苔藓（8.3%）。
眼部MMP患者通常有MMP的其他全身表现。最初表现为无眼部受累的眼外MMP患者有发生眼部疾病的风险。眼部MMP和类天疱疮样病变的临床特征相似；因此，需要对活检组织进行免疫组织学评估以确诊MMP。
