Chronic ulcerative stomatitis (Q43138): Difference between revisions
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Estomatite ulcerativa crônica (EUC) é uma doença rara que se apresenta com erosões e ulceração crônicas da mucosa oral semelhantes ao líquen plano erosivo, mas refratárias às terapias padrão. Tipicamente afeta mulheres idosas. O diagnóstico requer a demonstração de depósitos perinucleares granulares pontilhados de IgG nas camadas basal e parabasal do epitélio oral (antinuclear estratificado específico epitelial ou padrão SES-ANA). Há evidências crescentes de que a EUC é uma doença autoimune provocada por anticorpos IgG circulantes dirigidos contra delta-Np63, uma isoforma da família p53 de fatores de transcrição nuclear que está presente ao redor dos núcleos de queratinócitos basais epiteliais orais normais. A EUC normalmente responde a tratamento com hidroxicloroquina. | |||
| description / en | description / en | ||
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. | |||
Revision as of 09:59, 13 August 2026
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.
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | DA01.12 |
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| English | Chronic ulcerative stomatitis |
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. |
