The oral mucosa, or lining of the mouth, is one of the most common targets in lupus erythematosus (LE) patients. Internists, rheumatologists, and even dermatologists commonly omit examination of the mucous membrane when taking a patient's medical history and during the physical examination. This medical oversight may certainly delay appropriate medical care for LE patients, as LE mucosal lesions often give clues for a correct diagnosis and prognosis. In this article we try to give a practical yet comprehensive overview of the most common mucosal lesions found in LE.
SPECIFIC MUCOSAL LESIONS IN LE
Oral apthae (canker sores)
In the medical literature, oral apthae often are referred to as recurrent apthous stomatitis. These sores, or lesions, affect up to 15 percent of the normal population.
Conditions associated with a higher frequency of oral apthae are:
systemic lupus erythematosus (SLE)
inflammatory bowel disease (IBD)
acquired immune deficiency syndrome (AIDS) and other causes of immunodeficiency states
Behcet's Disease. Behcet's is a rare disease characterized by oral, genital, and skin ulcers as well as eye inflammation and systemic vasculitis.
Description: The oral apthae lesions are often small (less than 1 cm), painful, and have a tendency to occur on the buccal mucosae (inner cheeks) of the mouth. The lesions tend to last up to two to three weeks. The main mimicker of apthae is herpes (a.k.a. fever blisters). In herpes, the ulcers often affect the lips and gums. They tend to appear in groups and to be preceded by fluid-filled blisters.
Oral apthae in LE patients tend to last longer, be larger, and appear most often on the hard palate, or roof of the mouth. The most important feature of apthae in LE patients is the strong association of these oral ulcers with an internal organ flare of the systemic lupus disease.
Treatments: The most effective treatment for LE apthae is to control the SLE with a safe and immunosuppressive therapy. This usually is based on the combination of systemic corticosteroids with anti-metabolites, such as azathioprine (Imuran) or mycophenolate mofetil (CellCept) with cyclophosphamide.
Additional treatment may consist of high-potency corticosteroids placed directly upon the skin, such as clobetasol gel (4-5 times a day) or topical tacrolimus ointment (2-3 times a day); or medicines taken by mouth, such as colchicine, 0.6 mg twice a day, Dapsone, 100-150 mg/day, or thalidomide (Thalomid), 100-200 mg/day.
Mucosal discoid lupus erythematosus
Discoid lupus erythematosus (DLE) is the most common form of chronic cutaneous lupus erythematosus. The head and neck are most commonly affected areas. Very few people with DLE have associated systemic LE.
However, certain subsets of people DLE have a stronger relationship to systemic lupus. These are people with:
disseminated DLE (DLE lesions above and below the neck);
palmoplantar DLE (DLE lesion on the palms of the hands and soles of the feet);
familial DLE or familial SLE (first-degree relatives with DLE or SLE);
mucosal DLE (DLE lesions affecting mouth and rarely, other mucous membranes).
Description: DLE of the oral mucosa occurs only in the setting of cutaneous DLE. The most commonly affected location is the inner cheeks, and often there will be associated lip lesions. Usually these will be asymptomatic (presenting no symptoms), but when ulcerated they become quite painful.
The lesions resemble red plaques surrounded by lacy whitish areas. Mucosal DLE lesions are much like lesions seen in a very common disease called lichen planus. The presence of DLE-associated lesions on the skin and lips should prompt to the exclusion of oral DLE in patients with "lichen planus-like" mouth lesions.
Treatments: Treatment of mucosal DLE should be based on a combination of topical and systemic therapy. Topical therapy consists of high potency corticosteroids (clobetasol gel 4-5 times a day), with or without topical tacrolimus ointment (2-3 times a day). Thalidomide 100-200 mg daily, with or without hydroxychloroquine (Plaquenil) 200 mg twice daily, is often highly effective for oral DLE.
Rare severe cases may require systemic immunosuppressive drugs such as azathioprine. mycophenolate mofetil, or leflunomide (Arava).
Bullous SLE
This is a serious (fortunately, rare) disease in which individuals have antibodies against their own mouth and skin. These autoantibodies react against type VII collagen, a molecule found in the basement membrane zone. The basement membrane zone is an area where the outer layer of the skin (epidermis) and mouth (epithelium) are separated from the inner layer of skin (dermis) and mouth (submucosae).
Skin or mucosal biopsy and the evaluation of tissue-bound and circulating anti-type VII antibodies in the blood are the diagnostic procedures.
Description: Affected individuals generally have skin lesions consisting of grouped blisters, especially on the head and neck. The arms and legs also may be involved. Half of these individuals have extensive superficial erosions affecting the mouth, esophagus, larynx, trachea, genitalia, and eyes.
Treatments: Effective treatment consists of a combination of systemic corticosteroids and immunosuppressive therapy with agents such as azathioprine and/or mycophenolate mofetil. Severe cases or cases affecting the esophagus (food pipe), laryngotrachea (air pipe) mucosa or the eyes will require treatment with cyclophosphamide.
NON-SPECIFIC MUCOSAL LESIONS IN LE
These lesions often occur secondary to immunosuppressive or other therapies that LE patients are receiving.
Herpes simplex labialis (fever blisters)
Description: This type of herpes, which affects the mouth, is quite common in normal people. The sores appear as painful skin erosions, often preceded by small groups of fluid-filled blisters on the lips and gums. Episodes often last two to four weeks, but clear up without medication.
In normal populations, these breakouts occur about three to five times a year, and often are triggered by infections, stress, sun exposure and menstrual periods. People who have LE or are receiving immunosuppressive therapy tend to experience these outbreaks more often-even as frequently as every month-and the outbreaks tend to last longer than three weeks.
Treatments: Because systemic corticosteroid medications are the most common culprits for this complication in people with LE, the best ways to treat and prevent recurrent herpes is to lessen the exposure to such drugs in these individuals, and to use corticosteroid-sparing drugs (for example, azathioprine, mycophenolate mofetil, and cyclophosphamide) early in the course of disease.
However, some people with LE may require herpes treatment with antiviral drugs such as valacyclovir (Valtrex) or famciclovir (Famvir).
Stevens-Johnson Syndrome (SJS)
This disease is one of the rare complications of mucosal herpes outbreaks. Like herpes, SJS is triggered by medications; the most common are sulfa drugs, anticonvulsants, and pain killers. (SJS is known by many other names, such as Dermatostomatitis, Stevens Johnson Type; for additional information visit The National Organization for Rare Disorders website at http://www.rarediseases.org, or the Stevens Johnson Syndrome Foundation at http://www.sjsupport.org.
Description: People with SJS will show extensive ulcers in the eyes, mouth, nose, genitalia, and skin, usually two to four weeks after the herpes outbreak. The skin lesions are called "targets" because of the annular (ring) configuration. When these lesions coalesce, or merge, such extensive erosion occurs that affected individuals often must be admitted to a hospital burn unit.
Because of the potential exposure to the aforementioned drugs and their possible higher incidence of recurrent mucosal herpes, people with LE certainly would be more susceptible to SJS than the normal population.
Treatments: There is no effective treatment of SJS. The use of high doses of systemic corticosteroids in SJS could be associated with higher mortality due to infections. In some cases, individuals with SJS develop permanent and often debilitating scarring of the eyes.
Oral candidiasis
Oral candidiasis, also known as thrush, is a common complication of immunosuppressive therapy such as systemic corticosteroids. Individuals at a higher risk for recurrent or persistent oral thrush are:
immunodeficient
inhaler-dependent asthmatics
either type 1 or type 2 diabetics
suffering from AIDS.
Description: Thrush appears as whitish-red, flaky plaques that can affect any area of the mouth, and often involve the esophagus. The lesions are often asymptomatic, but patients may complain of a burning sensation and difficulty in swallowing.
Treatments: Prednisone dose reduction, Nystatin oral lozenges or pills, and the occasional use of oral antifungals such as fluconazole (Diflucan), are necessary to treat this disease.
Oral cancer
Immunosuppressed individuals, including people with LE who are receiving immunosuppressive therapy, are at higher risk for skin and mucosal cancer. One type of cancer in particular, called squamous cell carcinoma, is highly associated with human papilloma virus (HPV).
In these individuals, the disturbed immune system leads to an increased susceptibility to HPV (HPV also is the cause of common warts). Some types of HPV are precancerous; as warts, they have the potential to be transformed into squamous cell carcinomas that will affect the skin and oral and genital linings.
Description: Physicians must recognize the importance of biopsying lesions in the mucous membranes, such as early warty lesions, white plaques (precancerous leuokoplaquia) or red plaques (erythroplaquia). Especially important to identify are lesions located on the back of the inner cheeks and on the tongue.
Treatments: The treatment of precancerous warts is based on destructive methods, including surgery, electrocautery, and freezing. The topical skin cream imiquimod (Aldara) may bring additional benefits.
Established oral cancer often requires extensive surgical removal with radiation or chemotherapy. The best way to prevent this complication in LE patients is the appropriate use of immunosuppressive therapy.
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