Brydak-Godowska Joanna, Moneta-Wielgoś Joanna, Pauk-Domańska Magdalena, Dróbecka-Brydak Ewa, Samsel Agnieszka, Kecik Mariusz, Kowalewski Cezary, Mackiewicz Wojciech, Kecik Dariusz
Z Katedry i Kliniki Chorób Oczu Akademii Medycznej w Warszawie.
Klin Oczna. 2005;107(10-12):725-7.
Ocular cicatricial pemphigoid (OCP) is an autoimmune disease characterize by mucous membrane fibrosis and skin changes resulting with scarring. The pathogenic mechanisms of ocular cicatricial pemphigoid are incompletely understood. Antibasement membrane antibodies which lead to subepithelial blistering, granulation tissue and inflammatory infiltrate formation in the substantia propria are thought to be the main pathophysiological mechanisms in cicatricial pemphigoid. It has been found eosinophils and increased collagen type I and III. Human leukocyte antigen HLA-DR2, HLA-DR4 and DQw7 genotypes have been identified as conferring increased susceptibility to the development of this disease. Ocular cicatrical pemphigoid (OCP) is one of the forms of bullous pemphigoid. Initial symptoms of ocular pemfigoid are not characteristic. Conjunctival fibrosis may cause severe entropion, trichiasis, symblepharon, dry eye syndrome, corneal epithelial erosions or ulceration. Secondary glaucoma is one of the most frequent complications. Ocular cicatricial pemphigoid may be chronic, acute, or subacute disease with periodic exacerbation of conjunctival inflammation. The treatment in this disease are topical drops or ointment (lubricants, corticosteroids, antibiotics, antiglaucomatous). Oral dapsone and corticosteroids may control the activity of the disease. In other progressive cases immunosuppressive drugs must be used (azathioprine, cyclophosphamide, methotrexate, mycophenolan mofetil, daclizumab, intravenous immunoglobulin therapy). To make an early diagnosis of ocular cicatricial pemphigoid, biopsy and immunohistochemical analysis of conjunctiva should be performed in every case of persistent conjunctival inflammation.
瘢痕性类天疱疮(OCP)是一种自身免疫性疾病,其特征为黏膜纤维化和皮肤改变并导致瘢痕形成。瘢痕性类天疱疮的致病机制尚未完全明确。导致上皮下水疱形成、固有层肉芽组织和炎症浸润的抗基底膜抗体被认为是瘢痕性类天疱疮的主要病理生理机制。已发现嗜酸性粒细胞以及I型和III型胶原蛋白增加。人类白细胞抗原HLA - DR2、HLA - DR4和DQw7基因型已被确定为使个体对该疾病的发生易感性增加。眼部瘢痕性类天疱疮(OCP)是大疱性类天疱疮的一种形式。眼部类天疱疮的初始症状不具有特征性。结膜纤维化可导致严重睑内翻、倒睫、睑球粘连、干眼综合征、角膜上皮糜烂或溃疡。继发性青光眼是最常见的并发症之一。瘢痕性类天疱疮可能是慢性、急性或亚急性疾病,结膜炎症会周期性加重。该疾病的治疗方法是局部滴眼剂或眼膏(润滑剂、皮质类固醇、抗生素、抗青光眼药物)。口服氨苯砜和皮质类固醇可控制疾病活动。在其他进行性病例中,必须使用免疫抑制药物(硫唑嘌呤、环磷酰胺、甲氨蝶呤、霉酚酸酯、达利珠单抗、静脉注射免疫球蛋白疗法)。为早期诊断瘢痕性类天疱疮,对于每一例持续性结膜炎症患者均应进行结膜活检和免疫组织化学分析。