DA01.12ICD-11 MMS 2026-01
Chronic ulcerative stomatitis
Description
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.
Index terms
- Chronic ulcerative stomatitis
- CUS - [chronic ulcerative stomatitis]
Exclusions from above levels
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DA0C.31– Noma01– Certain infectious or parasitic diseases02– Neoplasms05– Endocrine, nutritional or metabolic diseases06– Mental, behavioural or neurodevelopmental disorders18– Complications of pregnancy, childbirth and the puerperium22– Injury, poisoning or certain other consequences of external causes
Sibling codes
DA01.10– Oral aphthae or aphtha-like ulcerationDA01.11– Oral mucositisDA01.13– Erythema multiforme with oral ulcerationDA01.14– Drug-induced oral ulcerDA01.15– Mouth ulcersDA01.1Y– Other specified noninfectious erosive or ulcerative disorders of oral mucosaDA01.1Z– Noninfectious erosive or ulcerative disorders of oral mucosa, unspecified
Foundation entity
Chronic ulcerative stomatitis – http://id.who.int/icd/entity/153761635
Part of: DA01.1: Noninfectious erosive or ulcerative disorders of oral mucosa › DA01: Disorders of oral mucosa › 13: Diseases of the digestive system