Chronic ulcerative stomatitis: Diagnostic and management challenges—four new cases and review of literature

Chronic ulcerative stomatitis (CUS) is a recently described mucocutaneous disease characterized by involvement of mucosal surfaces and skin. The disease usually presents in the form of painful oral ulcers and has been seen predominantly among older women. Clinically, CUS patients may exhibit erosive or ulcerative lesions of oral mucosa resembling erosive lichen planus and/or other vesiculobullous lesions. Routine histology may exhibit features of lichenoid mucositis and is often nondiagnostic. Direct immunofluorescence studies of mucosal or skin biopsies usually reveal a unique pattern of IgG bound to nuclei of keratinocytes of the basal and parabasal cell layers. Chronic ulcerative stomatitis exhibits a unique resistance to standard treatments available, including topical and systemic corticosteroids and immunomodulatory medications. However, long-lasting favorable clinical responses may be achieved with hydroxychloroquine pharmacotherapy. We present the clinicoimmunopathologic findings of four cases of CUS and discuss the varied clinical, microscopic spectrum, and treatment for this chronic, debilitating, and often recalcitrant condition. A systematic review of the literature, including 35 previously reported cases, is presented.