What are the other Names for this Condition? (Also known as/Synonyms)

• CUS (Chronic Ulcerative Stomatitis)

What is Chronic Ulcerative Stomatitis? (Definition/Background Information)

• Chronic Ulcerative Stomatitis (CUS) is described as a mucocutaneous disorder of the oral cavity that is characterized by severe, painful, and recurrent ulcers. CUS is an immune-mediated condition that is almost only reported among middle-aged and older women
• The condition may be misdiagnosed as oral erosive lichen planus or other oral lichenoid lesions. Hence, an accurate diagnosis of Chronic Ulcerative Stomatitis is very important. Also, unlike other mouth ulcers, CUS resists treatment using conventional topical and oral steroids. The prognosis is dependent on a variety of factors; although in many, the condition is longstanding

Who gets Chronic Ulcerative Stomatitis? (Age and Sex Distribution)

• A vast majority of Chronic Ulcerative Stomatitis cases are reported in middle-aged and older women (mean age around 55-60 years)
• Extremely rarely, the condition is reported in young adult women, men, and children
• Worldwide, there is no racial or ethnic preference observed
• However, CUS is a very rare condition with only around 100 cases recorded in the medical literature. Among these, many cases have been reported from Europe

What are the Risk Factors for Chronic Ulcerative Stomatitis? (Predisposing Factors)

• The risk factors for Chronic Ulcerative Stomatitis are presently unknown
• The condition is almost exclusively seen in middle-aged and older women (mostly Caucasians)

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Chronic Ulcerative Stomatitis? (Etiology)

• The exact cause of development of Chronic Ulcerative Stomatitis (CUS) is currently unknown
• CUS is described as an immune-mediated condition, meaning it is the result of an abnormal immune system

What are the Signs and Symptoms of Chronic Ulcerative Stomatitis?

The signs and symptoms of Chronic Ulcerative Stomatitis may include:

• Appearance of painful ulcers in the mouth presenting soreness (stomatitis)
• Dry mouth, irritation, and speaking/eating difficulty is commonly noted
• Individuals are often unable to consume hot or cold foods/drinks
• The ulcers are superficial (involve only the top mucosal layers) and may arise on the buccal cavity (cheeks), gums, tongue, and tongue

In over 75% of the cases, the oral mucosal lesions are found at multiple sites within the mouth; some are observed to be bilateral. In nearly 20% of the cases, skin involvement is noted. Hence the condition is categorized as a mucocutaneous disorder.

How is Chronic Ulcerative Stomatitis Diagnosed?

A diagnosis of Chronic Ulcerative Stomatitis (CUS) may involve the following exams and procedures. The diagnosis may be challenging as it presents many features similar to erosive oral lichen planus and lichenoid lesions of the mouth. Also, it is observed that many lab facilities are not well-equipped to perform direct immunofluorescence studies, which is crucial in establishing a diagnosis of CUS. In such a situation, tissue samples may require to be mailed to a reference laboratory.

• Physical examination, assessment of signs and symptoms, and medical history evaluation
• Oral cavity examination
• Culture studies
• Direct immunofluorescence (DIF) study: This study is important in establishing a diagnosis of CUS due to its similarity with oral lichen planus and oral lichenoid lesions (which it is often misdiagnosed as)
• Tissue biopsy: An oral tissue biopsy is performed and sent to a laboratory for a pathological examination, who examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. A biopsy is performed to rule out malignancies and other similar conditions

Major and minor diagnostic criteria have been proposed for Chronic Ulcerative Stomatitis:

Major diagnostic criteria:

• Erosive or exfoliative lesions in the mouth
• Characteristic indirect and direct immunofluorescence (IIF and DIF)

Minor diagnostic criteria:

• Chronic course with relapses
• Female in the older age group
• Response to hydroxychloroquine alone or combined with small doses of cortisone

A differential diagnosis is important to exclude other conditions that present similar signs and symptoms. These may include:

• Cicatricial pemphigoid
• Dermatitis herpetiformis
• Epidermolysis bullosa acquisita
• Erythema multiforme
• Lichenoid drug reaction
• Lichenoid stomatitis
• Linear IgA disease
• Oral erosive lichen planus
• Pemphigus vulgaris
• Pyostomatitis vegetans

Note: Unlike oral lichen planus and oral lichenoid lesions, CUS does not respond well to corticosteroid therapy. Hence, an accurate diagnosis of this ulcerative lesion is even more important.

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Chronic Ulcerative Stomatitis?

The possible complications of Chronic Ulcerative Stomatitis include:

• Persistently painful lesions resulting in severe emotional and psychological stress
• Sleeplessness
• Anxiety and depression
• Severe eating difficulties; nutritional imbalance due to poor eating
• Weight loss
• Superimposed bacterial and fungal infections
• Recurrence of the condition
• Poor response to therapy
• Treatment complications and side effects: The side effects of hydroxychloroquine may be severe if it is not administered carefully. Retinal damage due to the treatment may be irreversible

How is Chronic Ulcerative Stomatitis Treated?

Chronic Ulcerative Stomatitis (CUS) is a persistent and challenging condition to treat. Unlike many mouth ulcers that respond well to steroid therapy, it is not possible to manage CUS using topical and systemic corticosteroids. Also, the condition is resistant to treatment with immunomodulatory medications.

• Following a diagnosis of CUS, the ulcerous lesions respond well to oral hydroxychloroquine therapy, during the initial stages. However, the usage of hydroxychloroquine over a long duration of time may produce variable results, particularly after an initial remission period
• In such cases, the healthcare provider may prescribe low-dose corticosteroid along-with the antibiotic dapsone
• Maintaining good oral hygiene, dental care, and regular visits to a dentist office is recommended
• Providing reassurance and helping with feelings of stress and anxiety

A few self-care tips and home remedies for Chronic Ulcerative Stomatitis may include:

• Avoiding spicy and hot foods
• Avoiding foods that take an effort to bite and chew
• Using soft toothbrushes
• Drinking lots of water or fluids

Regular follow up visits with the healthcare provider is important and recommended since the condition can last for many years.

How can Chronic Ulcerative Stomatitis be Prevented?

• Currently, there are no specific methods or guidelines to prevent Chronic Ulcerative Stomatitis
• A careful and periodic monitoring or follow-up of the condition is recommended

What is the Prognosis of Chronic Ulcerative Stomatitis? (Outcomes/Resolutions)

• The prognosis of Chronic Ulcerative Stomatitis depends on the severity of the signs and symptoms, but it is generally good with appropriate treatment
• However, in many, the condition can be debilitating with severe and long-lasting symptoms that show periods of remission and relapse, and unfavorable response to conventional therapies

Additional and Relevant Useful Information for Chronic Ulcerative Stomatitis:

The following DoveMed website link is a useful resource for additional information:

https://www.dovemed.com/healthy-living/dental-health/