Lavallée Andréane, Bourret-Massicotte Dominique, Laughrea Patricia Ann
Centre Universitaire d'Ophtalmologie, Hôpital du Saint-Sacrement, CHU de Québec, Québec, Canada.
Cornea. 2013 Oct;32(10):1399-401. doi: 10.1097/ICO.0b013e3182a0ce6e.
To report a case of mucous membrane pemphigoid (MMP) occurring in childhood with exclusively ocular involvement.
Case report.
A 12-year-old girl presented with mild bilateral conjunctival cicatrization, symblepharon, and peripheral inflammatory keratitis. No other mucosal or cutaneous involvement was present. The findings of an initial conjunctival biopsy with immunofluorescence study were negative for MMP. After a few recurrences of mild corneal and conjunctival inflammation, a second conjunctival biopsy was performed at a different site in the same eye, 30 months later. Only then was the diagnosis of MMP confirmed using positive direct immunofluorescence. The results of a systemic investigation for autoimmune vasculitis were negative. Therapy with dapsone was introduced, and a good response was obtained.
MMP is an extremely rare condition in children. This case is the second one reported with exclusively ocular involvement. Despite its rarity in the pediatric age group, MMP should be considered in the differential diagnosis of chronic conjunctival inflammation and fibrosis. Furthermore, a single negative biopsy should not exclude this diagnosis in a suspected clinical case, even in children. When a high degree of suspicion is present, a repeat biopsy may prove useful.
报告一例仅累及眼部的儿童黏膜类天疱疮（MMP）病例。
病例报告。
一名12岁女孩出现双侧轻度结膜瘢痕形成、睑球粘连和周边炎性角膜炎。未发现其他黏膜或皮肤受累情况。初次结膜活检及免疫荧光研究结果显示MMP为阴性。在轻度角膜和结膜炎症反复出现几次后，30个月后在同一只眼的不同部位进行了第二次结膜活检。直到那时，通过阳性直接免疫荧光才确诊为MMP。自身免疫性血管炎的全身检查结果为阴性。开始使用氨苯砜治疗，获得了良好的反应。
MMP在儿童中极为罕见。该病例是第二例报告的仅累及眼部的病例。尽管在儿童年龄组中罕见，但在慢性结膜炎症和纤维化的鉴别诊断中应考虑MMP。此外，即使在儿童中，单次活检阴性也不应排除疑似临床病例中的该诊断。当高度怀疑时，重复活检可能有用。
