Chronic ulcerative stomatitis: Clinical, histopathologic, and immunopathologic findings

Chronic ulcerative stomatitis (CUS) is a mucocutaneous disease primarily involving mucosal surfaces, but occasionally may involve the skin. Clinically, CUS patients exhibit erosive or ulcerative lesions of the oral mucosa that resemble erosive oral lichen planus. Direct immunofluorescence (DIF) studies of mucosal or skin biopsies reveal a unique pattern of IgG immunoglobulin bound to nuclei of keratinocytes of the basal and lower one third cell layers, the stratified epithelial specific (SES) antinuclear antibody (ANA) pattern. Patient sera also exhibit circulating SES-ANA reactions on indirect immunofluorescence (IIF) using an esophagus substrate. We report the clinical and immunopathologic findings of 3 cases of CUS and demonstrate autoantibody recognition of the CUS antigen on Western blot. An important reason to distinguish CUS from other oral ulcerative conditions is that it may be refractory to standard treatments with topical corticosteroids, and favorable clinical responses may be achieved with hydroxychloroquine pharmacotherapy.