Chronic ulcerative stomatitis - Oral Mucosal Lesion
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Synopsis
Chronic ulcerative stomatitis (CUS) is an ulcerative condition of the oral cavity with clinical and histologic similarities to oral lichen planus (OLP).
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.
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.
Codes
ICD10CM:
K12.1 – Other forms of stomatitis
SNOMEDCT:
707268007 – Chronic ulcerative stomatitis
K12.1 – Other forms of stomatitis
SNOMEDCT:
707268007 – Chronic ulcerative stomatitis
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Last Reviewed:10/05/2022
Last Updated:10/12/2022
Last Updated:10/12/2022