Williams D M
Department of Oral Pathology, London Hospital Medical College, England.
J Oral Pathol Med. 1990 Jan;19(1):16-23. doi: 10.1111/j.1600-0714.1990.tb00776.x.
Bullous pemphigoid (BP) and benign mucous membrane pemphigoid (BMMP) are autoimmune diseases characterised by subepithelial bulla formation and showing substantial overlap in clinical signs and symptoms. BP principally involves skin and BMMP the oral mucosa and eyes. The gingiva are affected in 90% of cases of BMMP and buccal mucosa and palate in up to 30%. Lesions may heal with scarring. Extension into the pharynx and esophagus causes sore throat and dysphagia. Severe ocular involvement may cause blindness. Bulla formation is attributed to complement activation, following IgG binding to the basement membrane zone, with subsequent polymorphonuclear leukocyte accumulation. The target antigen in BP is a 180-230 kD protein associated with the basilar membrane of basal keratinocytes. The gene encoding the BP antigen has been partially cloned. It is likely that the same antigen is involved in BMMP, but the mechanism of scarring is not understood. Treatment of BP and BMMP includes systemic steroid and azathioprine therapy and topical steroids.
大疱性类天疱疮（BP）和良性黏膜类天疱疮（BMMP）是自身免疫性疾病，其特征为上皮下疱形成，在临床体征和症状上有大量重叠。BP主要累及皮肤，而BMMP主要累及口腔黏膜和眼睛。90%的BMMP病例牙龈受累，高达30%的病例颊黏膜和腭受累。病变愈合后可能会留下瘢痕。病变延伸至咽部和食管会导致咽痛和吞咽困难。严重的眼部受累可能导致失明。疱的形成归因于IgG与基底膜带结合后补体激活，随后有多形核白细胞积聚。BP中的靶抗原是一种与基底角质形成细胞基底膜相关的180 - 230 kD蛋白。编码BP抗原的基因已被部分克隆。BMMP可能涉及相同的抗原，但瘢痕形成的机制尚不清楚。BP和BMMP的治疗包括全身用类固醇和硫唑嘌呤治疗以及局部用类固醇。
