Pauk Sania Vidas, Geber Antonela, Bešlić Iva, Lakoš-Jukić Ines, Kuzman Tomislav
Department of Ophthalmology, University Hospital Centre Zagreb, School of Medicine, University of Zagreb, 10000 Zagreb, Croatia.
Department of Dermatovenereology, University Hospital Centre Zagreb, School of Medicine, University of Zagreb, 10000 Zagreb, Croatia.
Reports (MDPI). 2025 Jul 20;8(3):115. doi: 10.3390/reports8030115.
: Ocular cicatricial pemphigoid (OCP) is a rare autoimmune disease affecting the conjunctiva and oral mucosa. Chronic inflammation causes conjunctival scarring, leading to symblepharon, trichiasis, corneal damage, and possible blindness. Diagnosis is clinical, supported by biopsy and immunofluorescence. Treatment includes systemic corticosteroids, immunosuppressants, and biologics in refractory cases. : A 64-year-old male presented with ocular irritation, trichiasis, and counting fingers (CF) visual acuity in the left eye. Slit-lamp examination revealed conjunctival inflammation, corneal epithelial defect, and symblepharon in the left eye. Biopsy confirmed ocular cicatricial pemphigoid (OCP). He was treated with topical steroids, cyclosporine, subconjunctival injections, and systemic corticosteroids, followed by surgery, which improved BCVA to 0.10 logMAR. Two years later, disease progression resulted in severe inflammation and visual decline in both eyes. Systemic azathioprine and corticosteroids achieved partial control. Due to insufficient response, rituximab therapy was initiated, leading to significant reduction in inflammation and stabilization of disease. Right eye BCVA improved to 0.16 logMAR; the left remained at CF. The patient continues to receive rituximab during exacerbations and is under regular follow-up. : Early diagnosis and timely systemic treatment are essential in preventing vision loss in OCP. In refractory cases, biologic agents like rituximab may offer effective disease control.
眼部瘢痕性类天疱疮（OCP）是一种罕见的自身免疫性疾病，可累及结膜和口腔黏膜。慢性炎症会导致结膜瘢痕形成，进而引起睑球粘连、倒睫、角膜损伤，并可能导致失明。诊断主要依靠临床症状，活检和免疫荧光检查可提供支持。治疗方法包括全身使用皮质类固醇、免疫抑制剂，难治性病例可使用生物制剂。
一名64岁男性因左眼眼部刺激、倒睫以及仅能数指（CF）的视力而就诊。裂隙灯检查发现左眼结膜炎症、角膜上皮缺损和睑球粘连。活检确诊为眼部瘢痕性类天疱疮（OCP）。他接受了局部类固醇、环孢素、结膜下注射以及全身皮质类固醇治疗，随后进行了手术，最佳矫正视力（BCVA）提高到了0.10 logMAR。两年后，疾病进展导致双眼严重炎症和视力下降。全身使用硫唑嘌呤和皮质类固醇实现了部分控制。由于反应不足，开始使用利妥昔单抗治疗，炎症显著减轻，病情得到稳定。右眼BCVA提高到0.16 logMAR；左眼仍为CF。患者在病情加重期间继续接受利妥昔单抗治疗，并定期接受随访。
早期诊断和及时的全身治疗对于预防OCP导致的视力丧失至关重要。在难治性病例中，像利妥昔单抗这样的生物制剂可能有效控制疾病。
