Chronic ulcerative stomatitis K12.1

Jaremko et al. 1990

Chronic ulcerative stomatitis is a rare (possibly more frequently misdiagnosed than aphthae ) mucocutaneous disease characterized by the recurrent occurrence of extensive erosions and ulcerations of the oral mucosa (Qari H et al. 2015).

Almost exclusively women between the ages of 50 and 60 are affected (Reddy R et al. 2018).

Negative ANA diagnostics on Hep-2 cells. Positive nuclear fluorescence is found when using monkey esophagus.

The 70kDa transcription factor Delta Np63alpha, which is expressed by basal and suprabasal epithelial cells in multilayer squamous epithelium, could be detected as antigen. Delta Np63alpha is a regulator of epithelial stem cells and regulates the adhesion of basal and suprabasal keratinocytes.

Lichenoid mucositis with detection of IgG autoantibodies with a granular fluorescence pattern of the nuclei of basal and suprabasal keratinocytes (Reddy R et al. 2018). These are the "stratified epithelium-specific antinuclear antibodies (SES-ANA)" typical (but not specific) for CUS, which are also detected in a high percentage in oral lichen planus (Cozzani E et al. 2008).

A good response to hydroxychloroquine is described.

Some authors do not consider the clinical picture as an entity, but as a variant of the erosive oral lichen planus (Feller L et al. 2017).

1. Cozzani E et al. (2008) Patients with orally erosive and cutaneous lichen planus may have antibodies directed against the chronic ulcerative stomatitis protein antigen of 70-kDa. Acta Dermatovenerol Alp Pannonica Adriat 17:120-124.
2. Feller L et al. (2017)Is chronic ulcerative stomatitis a variant of lichen planus, or a distinct disease? J Oral Pathol Med 46:859-863.
3. Fourie J et al (2011) Chronic ulcerative stomatitis: a distinct clinical entity? SADJ 66:119-121.
4. Ko EM et al (2018) Chronic ulcerative stomatitis: case series of an under-recognized entity. J Cutan Pathol 45:927-932.
5. Qari H et al. (2015) The diagnostic challenges of separating chronic ulcerative stomatitis from oral lichen planus. Oral Surg Oral Med Oral Pathol Oral Radiol 120:622-627.
6. Reddy R et al (2018) Seventeen New Cases of Chronic Ulcerative Stomatitis with Literature Review. Head Neck Pathol doi: 10.1007/s12105-018-0982-7.