Is chronic ulcerative stomatitis an entity? Clinical and immunological findings in 18 cases

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.