Lugović Liborija, Buljan Marija, Situm Mirna, Poduje Sanja, Bulat Vedrana, Vucić Majda, Budimir Jozo
University Department of Dermatology and Venereology , Sestre milosrdnice University Hospital, Vinogradska cesta 29, HR-10000 Zagreb, Croatia.
Acta Dermatovenerol Croat. 2007;15(4):236-42.
Cicatricial pemphigoid is an autoimmune bullous disease characterized by mucous membrane fibrosis with resulting scarring, predominantly in the conjunctival and oral mucosa, which rarely involves skin changes. The majority of patients present with painful erosions or desquamative scarring gingivitis, resulting in eating and drinking disorders. Typical ocular lesions include chronic scarring conjunctivitis with progressive subconjunctival fibrosis, fornix foreshortening and synechia formation between the bulbar and palpebral conjunctiva, occasionally resulting in blindness. A 69-year-old woman was admitted to our Department for intense pain and severe burning sensation in the oral cavity, induced by several erosions and solitary blisters, lasting for 3 years. She was also diagnosed with the right eye symblepharon, lid entropion, trichiasis, leukoma and blindness of the right eye. The diagnosis of cicatricial pemphigoid was based on clinical picture and histopathology combined with immunofluorescence methods, with therapy initiated thereupon. Systemic corticosteroid (methylprednisolone) therapy in combination with azathioprine proved successful in the treatment of oral lesions as well as for stabilization of ocular lesions. Unfortunately, the patient was diagnosed in the advanced stage when scarring had already occurred. Prompt recognition of cicatricial pemphigoid and close patient monitoring are an imperative for the future prognosis of the disease.
瘢痕性类天疱疮是一种自身免疫性大疱性疾病，其特征为黏膜纤维化并导致瘢痕形成，主要累及结膜和口腔黏膜，很少累及皮肤。大多数患者表现为疼痛性糜烂或脱屑性瘢痕性牙龈炎，导致饮食障碍。典型的眼部病变包括慢性瘢痕性结膜炎伴进行性结膜下纤维化、穹窿缩短以及球结膜和睑结膜之间的粘连形成，偶尔会导致失明。一名69岁女性因口腔内多处糜烂和单个水疱引起的剧烈疼痛和严重烧灼感入院，症状持续3年。她还被诊断为右眼睑球粘连、睑内翻、倒睫、角膜白斑和右眼失明。瘢痕性类天疱疮的诊断基于临床表现、组织病理学以及免疫荧光方法，并随即开始治疗。全身用皮质类固醇（甲泼尼龙）联合硫唑嘌呤治疗在治疗口腔病变以及稳定眼部病变方面取得了成功。不幸的是，该患者在瘢痕已经形成的晚期才被诊断出来。对瘢痕性类天疱疮的及时识别和对患者的密切监测对该病的未来预后至关重要。
