School of Medicine, University of Puerto Rico Medical Sciences Campus, San Juan, PR.
Department of Ophthalmology, School of Medicine, University of Puerto Rico Medical Sciences Campus, San Juan, PR.
P R Health Sci J. 2020 Mar;39(1):34-38.
To describe the characteristics upon presentation of a cohort of Hispanic patients living in Puerto Rico with ocular mucous membrane pemphigoid (MMP).
Retrospective chart review of subjects with ocular MMP at one academic institution and one private practice. Patients with clinical evidence of ocular MMP, along with a positive mucous membrane biopsy revealing linear antibody or C3 deposition in the basement membrane zone, or with a positive indirect immunofluorescence assay were included. Descriptive statistical analysis was performed.
Eight patients with ocular mucous membrane pemphigoid were identified. The median age upon presentation was 60.5 years; however, 2 patients were in their 4th decade and one in the 5th decade of life. Females constituted 62.5% of the cohort. All patients presented with stage III ocular MMP in at least one eye and 50% had history of trichiasis. Seven out of eight patients (87.5%) had extraocular symptoms for a median duration of 36 months (range 2-144 months). The most common site of extraocular involvement was the oropharynx, present in 87.5% of patients.
Our results suggest that in Puerto Rico ocular MMP most commonly presents in the seventh decade of life. The presence of symblepharon, trichiasis or oropharyngeal mucosal disease should prompt further evaluation and consideration for immunopathological tissue analysis and an IIF assay.
描述波多黎各一群西班牙裔患者出现眼部黏膜类天疱疮 (MMP) 的特征。
对一家学术机构和一家私人诊所的眼部 MMP 患者进行回顾性病历审查。纳入标准为：具有眼部 MMP 的临床证据，以及黏膜活检显示基底膜带存在线性抗体或 C3 沉积，或间接免疫荧光检测阳性的患者。进行描述性统计分析。
共发现 8 例眼部黏膜类天疱疮患者。就诊时的中位年龄为 60.5 岁，但有 2 例患者年龄在 40 岁出头，1 例患者在 50 岁出头。女性占队列的 62.5%。所有患者至少一眼表现为 III 期眼部 MMP，50%有倒睫史。8 例患者中有 7 例（87.5%）有眼外症状，中位持续时间为 36 个月（范围 2-144 个月）。最常见的眼外受累部位是口咽部，占患者的 87.5%。
我们的结果表明，在波多黎各，眼部 MMP 最常发生在 70 多岁。出现睑球粘连、倒睫或口咽黏膜疾病时，应进一步评估并考虑进行免疫病理组织分析和 IIF 检测。
