ICD-11 code : DA01.12;
Preferred Label : Chronic ulcerative stomatitis;
ICD-11 definition : 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.;
ICD-11 synonym : CUS - [chronic ulcerative stomatitis];
ICD-11 acronym : CUS;
Origin ID : 153761635;
Automatic exact mappings (from CISMeF team)
- ICD-10 Mapping
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.
