• chronic ulcerative stomatitis;
  • oral lichen planus;
  • ΔNp63α;
  • autoantibodies

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, ΔNp63α, 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.