ICD-11 MMS

13 Diseases of the digestive system

Diseases or disorders of orofacial complex  (DA00-DA0Z)

DA01 Disorders of oral mucosa

DA01.1 Noninfectious erosive or ulcerative disorders of oral mucosa

DA01.12 Chronic ulcerative stomatitis

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01


Chronic ulcerative stomatitis (CUS) is a rare disease which presents with chronic oral mucosal erosions and ulceration resembling erosive lichen planus but refractory to standard therapies. It typically occurs in older women. Diagnosis requires the demonstration of speckled granular perinuclear IgG deposits in the basal and parabasal layers of oral epithelium (stratified epithelium-specific antinuclear or SES-ANA pattern). There is increasing evidence that CUS is an autoimmune disease provoked by circulating IgG antibodies directed against delta-Np63, an isoform of the p53 family of nuclear transcription factors which is present around the nuclei of normal oral epithelial basal keratinocytes. CUS normally responds to treatment with hydroxychloroquine.

synonyms

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