Chronic ulcerative stomatitis: a distinct clinical entity

Introduction: Chronic ulcerative stomatitis (CUS) is a mucocutaneous disorder which is characterised by persistent oral mucosal ulceration, and rarely skin changes. The clinical appearance resembles lichen planus (LP) and may thus result in an erroneous diagnosis. The immune mediated inhibition of the CUS protein (CUSP) is implicated in the pathogenesis of CUS. CUSP acts as an anti-apoptotic protein. When its action is prevented through auto-antibodies, it may result in significant epithelial injury. Objectives: To present a case of CUS with reference to international literature. Clinicians should be alerted to CUS as a diagnostic entity in patients who present with particularly ulcerative lichen planus type lesions. Methods: A case is presented in which the differential diagnosis of the condition is discussed. The use of direct immunofluorescence is presented as an essential aid in diagnosing CUS and differentiating the condition from other ulcerative conditions such as LP. Results: CUS should be diagnosed using direct immunofluorescence. The condition can be successfully managed using hydroxy/chloroquine or cyclosporine. The presented case is significant due to the extraordinary skin involvement which displays similarities with palmo-plantar lichen planus. Skin involvement is a rare finding in CUS. Conclusion: Due to the clinical and histological similarities between CUS and LP, the true prevalence of CUS is not known. Immunofluorescence should be considered in the diagnostic process of especially atrophic/ulcerative cases of clinically and routine histologically appearing LP. CUS is typically resistant to glucocorticosteroid therapy, but responds well to the anti-malaria therapy such as hydroxy/chloroquine. Additionally, cyclosporine may be considered for use.