Chronic ulcerative stomatitis: A comprehensive review and proposal for diagnostic criteria

Abstract

Chronic ulcerative stomatitis (CUS) is an immune‐mediated disorder characterized by oral erosions and ulcers usually refractory to conventional treatments. The disease often involves middle‐aged and older women with painful lesions sometimes resembling those of erosive oral lichen planus (OLP). The most affected sites are the buccal mucosa, the gingiva and the tongue, but the skin is involved in 22.5% of cases. Histopathologic features in CUS are non‐specific and indistinguishable from those of OLP, with the exception of the presence of a mixed infiltrate composed of lymphocytes and plasma cells. Direct immunofluorescence (DIF) analysis reveals the presence of stratified epithelium‐specific antinuclear antibodies (SES‐ANA) in the lower third of the epithelium. The IgG antibodies detected on DIF are directed against the ∆Np63α isoform of p63 expressed in the nuclei of the epithelial basal cells. A distinguishing feature of CUS is the low response to conventional corticosteroid therapy and the good outcome with hydroxychloroquine at the dosage of 200 mg/day or higher dosages. This paper presents a comprehensive review of CUS and is accompanied by a new case report (the 73rd case) and a proposal for updated diagnostic criteria.

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“…It was suggested for the treatment of primary Sjögrenʹs syndrome 84 , and was recommended for the treatment of chronic ulcerative stomatitis. 85 Interestingly, it was also suggested in the treatment of oral squamous cell carcinoma due to its role in cell protection by eliminating excessive proteins and injured/aged organelles in the microenvironment of tumors with subsequent acceleration of tumor cell death. 86 …”

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“…It was noticed to possess efficacy towards autoimmune diseases and has been implemented since the 1980s in the treatment of systemic lupus erythematosus (SLE), a disease that may have oral manifestations like ulcers. Its use in the treatment of primary Sjögren's syndrome has been suggested by some scientists [24], and it is also recommended for the treatment of chronic ulcerative stomatitis [25]. It had been suggested for the treatment of oral squamous cell carcinoma due to its role in cell protection by eliminating excessive proteins and injured/aged organelles in the microenvironment of tumors with subsequent acceleration of tumor cell death [26].…”

Novel combination therapy of hydroxychloroquine and topical tacrolimus for chronic ulcerative stomatitis

“…Because of similar clinical presentation to other conditions, such as oral lichen planus (OLP), diagnosis of CUS is challenging and often delayed 2 . CUS is most commonly observed in middle‐aged Caucasian females with mean age of onset and diagnosis at 57 and 62 years, respectively 2 . Etiopathogenesis is associated with binding of the immunoglobulin IgG to ΔNp63α, a nuclear protein located in the basal and parabasal layers of the stratified squamous epithelium, which results in separation of keratinocytes from each other and from the basement membrane 2 .…”

“…Chronic ulcerative stomatitis (CUS) is a rare immune‐mediated mucosal disorder characterized by symptomatic chronic oral ulcers not typically associated with cutaneous lesions 1 . Because of similar clinical presentation to other conditions, such as oral lichen planus (OLP), diagnosis of CUS is challenging and often delayed 2 . CUS is most commonly observed in middle‐aged Caucasian females with mean age of onset and diagnosis at 57 and 62 years, respectively 2 .…”

“…CUS is most commonly observed in middle‐aged Caucasian females with mean age of onset and diagnosis at 57 and 62 years, respectively 2 . Etiopathogenesis is associated with binding of the immunoglobulin IgG to ΔNp63α, a nuclear protein located in the basal and parabasal layers of the stratified squamous epithelium, which results in separation of keratinocytes from each other and from the basement membrane 2 . Clinical manifestations of CUS include Wickham’s striae, erythema, and ulceration which commonly affect the buccal mucosa and tongue.…”