Chronic ulcerative
stomatitis (
CUS) is a recently described condition with specific immunopathologic findings. Demographics indicate that white women in their late middle age are more susceptible to this condition. The clinical history of
CUS patients is of painful, exacerbating and remitting oral erosions, and ulcerations. The histologic features are non-specific, with a chronic inflammatory infiltrate, often appearing similar to
oral lichen planus (OLP). Diagnosis of
CUS requires surgical biopsy with immunofluorescence microscopic examination. Accurate diagnosis is important because the usual treatment option for immunologically mediated diseases,
glucocorticoids, is often not effective in treating
CUS. However,
hydroxychloroquine pharmacotherapy is beneficial in many cases. The lack of awareness of the condition among clinicians and the technical challenges in specimen processing make diagnosis of
CUS a challenge, and hence the true prevalence is unknown. Immunofluorescence studies show circulating and tissue-bound
autoantibodies to a
protein, DeltaNp63alpha, which is a normal component of stratified epithelia. It is unknown if the
antibodies are pathogenic, thus the etiology of
CUS is also unknown. Studies are needed to elucidate the pathogenesis of
CUS, optimize clinical management, and clarify its relationship to OLP and
neoplasia.