Chorzelski T P, Olszewska M, Jarzabek-Chorzelska M, Jablonska S
Department of Dermatology, Warsaw School of Medicine, Koszykawa 82a, 02-008 Warsaw, Poland.
Eur J Dermatol. 1998 Jun;8(4):261-5.
Chronic ulcerative stomatitis (CUS), a rare disease of oral mucosa found to be associated with unusual antinuclear antibodies reactive exclusively with squamous epithelia (squamous epithelium-specific antinuclear antibodies - SES-ANA), has been reported to occur almost exclusively in older females and to respond dramatically to antimalarials. We report on a large series of 18 cases positive for SES-ANA, 15 of them with active CUS, followed-up for several years. We found that the disease may occur, although rarely, in men and younger females. The antibodies persist after clearance of mucosal lesions, are fixed in vivo and also in uninvolved skin and other mucosa, and may occur in patients without CUS. Thus their pathogenic potential remains to be established. We also describe some unusual cases of CUS, one associated with cicatricial conjunctivitis, another associated with gluten-sensitive enteropathy and bizarre skin lesions, and 3 cases associated with lichen planus (LP) or LP-like lesions. The antimalarials, initially highly effective, even for several months or years, not infrequently proved to be insufficient and the relapses responded usually only to combined therapy with chloroquine and small doses of corticosteroids. SES-ANA antibodies are quite specific and present an immunologic marker of this unusual autoimmune disease.
慢性溃疡性口炎（CUS）是一种罕见的口腔黏膜疾病，被发现与仅对鳞状上皮有反应的异常抗核抗体（鳞状上皮特异性抗核抗体-SES-ANA）相关，据报道几乎仅发生于老年女性，对抗疟药有显著反应。我们报告了一系列18例SES-ANA阳性病例，其中15例患有活动性CUS，并进行了数年随访。我们发现该疾病虽罕见，但也可发生于男性和年轻女性。抗体在黏膜病变清除后持续存在，在体内以及未受累的皮肤和其他黏膜中均可固定，且可出现在无CUS的患者中。因此其致病潜力仍有待确定。我们还描述了一些CUS的特殊病例，1例与瘢痕性结膜炎相关，另1例与麸质敏感肠病及奇异皮肤病变相关，还有3例与扁平苔藓（LP）或LP样病变相关。抗疟药起初非常有效，甚至可持续数月或数年，但常常被证明并不充分，复发通常仅对氯喹和小剂量皮质类固醇的联合治疗有反应。SES-ANA抗体相当特异，是这种罕见自身免疫性疾病的一种免疫标志物。
