CHRONIC ULCERATIVE STOMATITIS: A LICHENOID OR VESICULOBULLOUS DISEASE?

Abstract

Objectives Chronic ulcerative stomatitis (CUS) is a rare disease of unknown etiology. The histopathologic features are similar to lichen planus, but direct immunofluorescence (DIF) studies show characteristic presence of IgG in basal and parabasal epithelial nuclei.This study will review a case series of CUS and assess if the entity is more similar to lichen planus or vesiculobullous diseases. Methods An IRB-approved retrospective search of CUS was performed within the archives of the UF Oral Pathology Biopsy Service between 2007 and 2017. Findings Seventeen cases, all female, were included. The median age was 64 years (range 47-83 years). Eleven patients were Caucasian, one was Asian, and one was African-American. Race was not specified in four cases. Buccal mucosa (8/17) was the most common location, followed by gingiva (7/17), buccal vestibule (1/17), and gingiva/buccal mucosa (1/17). The most common clinical presentations were pain/burning (13/17), erythema (13/17), whiteness (11/17), ulcerations/erosions (5/17), blisters/positive Nikolsky's sign (5/17), sloughing (2/17), striae (2/17), and recession (1/17). The clinical impression was lichen planus in 12 cases. Of these twelve cases, 4 included vesiculobullous disease as a differential. Four cases did not include a clinical impression and one listed erythema multiforme as the clinical impression. All cases were confirmed with DIF testing that showed a characteristic speckled pattern of IgG in basal and parabasal cells. Eleven of these cases were also positive for fibrinogen and two cases were faintly positive for C3. None of the cases were positive for IgA or IgM. Conclusion Since CUS has overlapping clinical, histological, and immunofluorescence features with lichen planus and vesiculobullous diseases, clinicians and pathologists should consider this unusual, but significant, entity whenever oral ulcerative diseases with mixed features are encountered.