Ghanbari Hamidreza, Rahimi Masoud, Momeni Ali, Aminizade Mehdi, Nozarian Zohreh, Moghtader Amirhossein, Rubinstein Daniel E, Basu Sayan, Sangwan Virender S, Djalilian Ali R, Soleimani Mohammad
Eye Research Center, Farabi Eye Hospital, Qazvin Sq, Tehran, Iran.
Department of Ophthalmology and Visual Science, Cornea Service, Eye and Ear Infirmary, University of IL, University of Illinois at Chicago, Chicago, IL, USA.
Graefes Arch Clin Exp Ophthalmol. 2025 Feb 5. doi: 10.1007/s00417-025-06756-2.
Mucous membrane pemphigoid (MMP) is a systemic autoimmune condition characterized by blistering and cicatrization, predominantly affecting mucous membranes, including those lining the esophagus, oropharynx, nasal cavity, trachea, conjunctiva, and genitalia. Ocular mucous membrane pemphigoid (OMMP) is observed in approximately 70% of MMP cases. This study aims to review the pathophysiology, clinical manifestations, diagnosis, treatment, and complications of OMMP.
A literature search was conducted using MEDLINE and EMBASE databases.
OMMP is characterized by the deposition of autoantibodies along the basement membrane zone of mucous membranes, particularly affecting the conjunctival epithelium. OMMP manifests as chronic ocular discomfort, inflammation, conjunctival scarring, eyelid abnormalities, and visual impairment. Given the extensive range of similar conditions, including drug-induced pseudo-pemphigoid and paraneoplastic conjunctival cicatrization, challenges in differential diagnosis may arise. The clinical diagnosis of OMMP is supported by confirmatory biopsy with histopathology and immunofluorescence studies. The mainstay of management includes systemic immunomodulatory medications and anti-inflammatory agents, tailored to disease severity. Surgical interventions may be necessary, although caution is warranted due to the risk of exacerbating OMMP. Prompt diagnosis and treatment are essential to halt disease progression and prevent vision loss. Complications of OMMP include corneal disorders, lid disorders, and vision disturbances. A comprehensive understanding of OMMP aids in timely intervention and improved patient outcomes.
OMMP is a bilateral, chronic, progressive, relapsing-remitting condition. Early diagnosis and treatment of OMMP are necessary to prevent disease progression. The management of OMMP varies according to the severity of the disease, but often involves both medical control of the underlying inflammatory process and subsequent surgical correction of residual anatomical changes.
黏膜类天疱疮（MMP）是一种全身性自身免疫性疾病，其特征为水疱形成和瘢痕化，主要累及黏膜，包括食管、口咽、鼻腔、气管、结膜和生殖器的黏膜。约70%的MMP病例会出现眼部黏膜类天疱疮（OMMP）。本研究旨在综述OMMP的病理生理学、临床表现、诊断、治疗及并发症。
使用MEDLINE和EMBASE数据库进行文献检索。
OMMP的特征是自身抗体沿黏膜基底膜带沉积，尤其影响结膜上皮。OMMP表现为慢性眼部不适、炎症、结膜瘢痕形成、眼睑异常和视力损害。鉴于存在广泛的类似病症，包括药物性类天疱疮样疹和副肿瘤性结膜瘢痕化，鉴别诊断可能存在挑战。OMMP的临床诊断需通过组织病理学和免疫荧光研究的确诊活检来支持。治疗的主要手段包括根据疾病严重程度调整的全身性免疫调节药物和抗炎药物。可能需要进行手术干预，不过由于存在加重OMMP的风险，需谨慎操作。及时诊断和治疗对于阻止疾病进展和预防视力丧失至关重要。OMMP的并发症包括角膜疾病、眼睑疾病和视力障碍。全面了解OMMP有助于及时干预并改善患者预后。
OMMP是一种双侧性、慢性、进行性、复发缓解性疾病。早期诊断和治疗OMMP对于预防疾病进展是必要的。OMMP的治疗根据疾病严重程度而异，但通常涉及对潜在炎症过程的药物控制以及随后对残余解剖学改变的手术矫正。
