Chronic ulcerative stomatitis - Oral Mucosal Lesion
The main autoantigen of CUS is a 70-kDa epithelial nuclear protein known as chronic ulcerative stomatitis protein (CUSP). CUSP plays a critically important role in the normal development of the oral epithelium and skin. In CUS, immunoglobulin G (IgG) antibodies and, less commonly, immunoglobulin A (IgA antibodies), known as stratified epithelium-specific antinuclear antibodies (SES-ANAs), are directed toward this protein.
CUS has a predominantly female distribution (female-to-male ratio of 14:1), with a mean age of 61 years and a median age of 64 years at diagnosis. CUS presents most often in White (91.3%) followed by Black, Hispanic, and Asian populations.
The most common signs of the disease include erosions, white lesions / striae, erythema, ulcerations, and leukoplakia. Xerostomia, vesiculation with positive gingival Nikolsky's sign, and gingival recession may be seen. These findings may be accompanied by pain, a burning / stinging sensation, and difficulty eating.
Rarely, ocular involvement and cutaneous involvement have been reported.
The clinical course of CUS is often relapsing and remitting.
K12.1 – Other forms of stomatitis
707268007 – Chronic ulcerative stomatitis
Differential Diagnosis & Pitfalls
- Oral lichen planus (OLP) – CUS is most commonly mistaken for OLP both clinically and histologically. Of note, striae on the buccal mucosa are reported to be more common in OLP than CUS. In OLP, there is also fibrinogen deposition at the basement membrane, causing a "shaggy" appearance on direct immunofluorescence (DIF), but in contrast to the mixed infiltrate of CUS, a pure T-lymphocyte infiltrate is seen in OLP. OLP is typically responsive to oral steroids.
- Lichenoid drug reaction
- Pemphigus vulgaris
- Systemic lupus erythematosus (SLE) – Oral ulcers are a feature.
- Discoid lupus erythematosus
- Bullous pemphigoid
- Mucous membrane pemphigoid