慢性溃疡性口炎

定义

慢性溃疡性口炎（CUS）是一种罕见病，表现为慢性口腔粘膜糜烂和溃疡，类似于糜烂性扁平苔藓，但对标准治疗无效。它通常发生在老年妇女身上。诊断需要在口腔上皮的基底层和副基底层显示出斑点粒状核周IgG沉积（分层上皮特异性抗核或SES-ANA模式）。越来越多的证据表明，CUS是一种由针对delta-Np63的循环IgG抗体引发的自身免疫性疾病，delta-Np63是核转录因子p53家族的一种异构体，存在于正常口腔上皮基底角质细胞的细胞核周围。CUS通常对羟氯喹治疗有效。

Chronic ulcerative stomatitis (CUS) is a rare disease which presents with chronic oral mucosal erosions and ulceration resembling erosive lichen planus but refractory to standard therapies. It typically occurs in older women. Diagnosis requires the demonstration of speckled granular perinuclear IgG deposits in the basal and parabasal layers of oral epithelium (stratified epithelium-specific antinuclear or SES-ANA pattern). There is increasing evidence that CUS is an autoimmune disease provoked by circulating IgG antibodies directed against delta-Np63, an isoform of the p53 family of nuclear transcription factors which is present around the nuclei of normal oral epithelial basal keratinocytes. CUS normally responds to treatment with hydroxychloroquine.

索引词 可用于检索该条目的同义词与别名

• 慢性溃疡性口炎
• CUS[慢性溃疡性口炎]