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

• Genital Mucosal Lupus Erythematosus
• MLE (Mucosal Lupus Erythematosus)
• Oral Mucosal Lupus Erythematosus

What is Mucosal Lupus Erythematosus? (Definition/Background Information)

• Mucosal Lupus Erythematosus (MLE) may be described as the involvement of mucosal membranes in lupus erythematosus. It is seen with Systemic Lupus Erythematosus (SLE) in some cases or may occur in the Discoid Lupus Erythematosus (DLE) subclass of the subtype Chronic Cutaneous Lupus Erythematosus (CCLE)
• Some researchers describe it as a subtype of chronic cutaneous lupus erythematosus that form a group of longstanding autoimmune disorders affecting primarily the skin. Autoimmune disorders cause the body to mistake normal healthy tissues and organs for dangerous foreign material and attack them leading to long-term chronic inflammation
• Mucosal Lupus Erythematosus may present painless red patches to ulcerous sores in the mouth (usually on the cheek or buccal mucosa), nasal cavity, or genital mucosal surfaces. The lesions frequently resemble lichen planus or other lichenoid reactions (such as due to an adverse drug reaction)
• Mucosal Lupus Erythematosus usually affects young and middle-aged women. The exact cause of MLE is not yet known. In many cases, it is associated with active systemic lupus erythematosus, which is a serious disorder that can potentially involve several organs and systems of the body
• The condition may be diagnosed through physical examination, complete medical history, and specialized blood tests. The treatment of Mucosal Lupus Erythematosus may involve topical steroidal mouth applications, oral medication, and systemic corticosteroids for severe symptoms
• The prognosis of Mucosal Lupus Erythematosus depends on the severity of the disorder and its response to therapy. It is also dependent upon the involved subtype of lupus. Individuals with skin (and mucosal membrane) symptoms have a better prognosis than when the entire body is involved

Who gets Mucosal Lupus Erythematosus? (Age and Sex Distribution)

• Individuals of any age may be affected by Mucosal Lupus Erythematosus; but it is more commonly seen in young and middle-aged adults
• Women are more prone to be affected than men
• Both fair-skinned and dark-skinned individuals can be affected, although darker-skinned Africans and Asians have a higher risk than fair-skinned Caucasians for MLE

What are the Risk Factors for Mucosal Lupus Erythematosus? (Predisposing Factors)

Risk factors associated with Mucosal Lupus Erythematosus may include:

• Presence of systemic lupus erythematosus (SLE), an autoimmune disorder affecting several systems and organs of the body. It is reported that many cases of MLE are associated with SLE and Discoid Lupus Erythematosus (DLE)
• Female gender
• Genetic predisposition: A family history of any of the forms of systemic or cutaneous lupus erythematosus, which may be related to HLA genetic makeup, may increase one’s risk

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 Mucosal Lupus Erythematosus? (Etiology)

The exact cause of Mucosal Lupus Erythematosus is not fully known or understood. It is seen as part of cutaneous or systemic lupus.

• It is most likely an autoimmune disorder, where antibodies of the body that fight diseases or infections attack one’s healthy tissues instead. Such an attack by the body’s immune cells against its tissue can be severe, resulting in significant signs and symptoms
• MLE is associated with systemic lupus erythematosus (SLE) in many cases. It may also occur when there is a severe flare-up of SLE. This can often result in inflammation in other parts of the body
• Some individuals may have a genetic predisposition for the condition; which may then be triggered by infections, certain drugs, or on exposure to sunlight

What are the Signs and Symptoms of Mucosal Lupus Erythematosus?

The signs and symptoms of Mucosal Lupus Erythematosus (MLE) may vary from one individual to another. It may be mild in some individuals or severe in others and may include:

• Presence of red lesions on the mucosal surfaces that appear to radiate from a central spot; a central red ulcer with a white halo and radiating markings may be observed inside the mouth
• The lesions are seen either as ulcers or plaques with scales; pain may or may not be present
• The ulcers can form on the genital mucosa, lips, mouth, eyelids, and inside the nasal cavities; lesions affecting the eye may lead to the loss of eyelashes
• Most of the ulcerous lesions inside the mouth are observed in the cheek, although any part of the mouth may be involved
• Oral cavity lesions are normally observed as part of an active lupus
• Rarely, in the genitalia, lesions may form on the glans penis or vulva
• Individuals with oral cavity lesions may have dry mouth (xerostomia), especially when the condition is associated with Sjögren Syndrome
• Other skin and systemic manifestations associated with lupus subtypes may be present

The signs and symptoms of systemic lupus erythematosus, if present, may be noted. Apart from the skin symptoms, this may include low-grade fever, appetite loss, joint pain, enlarged lymph nodes, and involvement of various body organs.

Certain factors can worsen the signs and symptoms (causing flare-ups), and these include:

• Sun exposure
• Trauma
• Smoking
• Certain viral infections
• Certain medications
• Hormonal influence

How is Mucosal Lupus Erythematosus Diagnosed?

Mucosal Lupus Erythematosus may be diagnosed using the following tests and exams:

• Complete physical examination with a comprehensive evaluation of one’s medical history
• Assessment of the signs and symptoms
• Dermoscopy: It is a diagnostic tool where a dermatologist examines the skin using a special magnified lens
• Wood’s lamp examination: In this procedure, the healthcare provider examines the skin using ultraviolet light. It is performed to examine the change in skin pigmentation
• Blood tests that include:
  • Routine blood testing (sedimentation rate/C-reactive protein)
  • Antinuclear antibody (ANA) levels blood test to look for antibodies in blood
  • Serological blood test for anti-double-stranded DNA (ds-DNA)
  • Extractable nuclear antigen antibodies panel blood test to detect anti-Ro antibodies and anti-La antibodies
  • Blood chemistry panels
• Body fluid analysis, if needed
• Skin/tissue biopsy: A tissue biopsy is performed and sent to a laboratory for a pathological examination. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed), and microscope findings, the pathologist arrives at a definitive diagnosis
• Direct immunofluorescence studies on tissue biopsies
• A differential diagnosis is undertaken to exclude lichen planus or drug reactions (such as caused by NSAIDs) that show symptoms similar to MLE

In order to diagnose systemic lupus erythematosus, a set of criteria established by the US-based organization American College of Rheumatology may be used.

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 Mucosal Lupus Erythematosus?

The complications of Mucosal Lupus Erythematosus may include:

• Secondary bacterial and fungal infection due to bleeding and ulceration
• If the eyes are involved, it may lead to loss of eyelashes and other cosmetic disfigurements
• Emotional and psychological stress
• Eating and chewing difficulties, if lesions involve the mouth
• Difficulty in having sex, if the genitalia is involved
• Severe systemic lupus erythematosus (SLE) causing involvement of vital organs, such as the brain, lungs, heart, liver, and kidneys, resulting in organ dysfunction
• Longstanding ulcers can result in the development of squamous cell carcinoma (SCC)

How is Mucosal Lupus Erythematosus Treated?

The treatment undertaken for Mucosal Lupus Erythematosus consists of decreasing inflammation and autoimmune activity in the body and minimizing the occurrence of flare-ups. The treatment measures may include:

• Topical steroidal mouthwashes
• Topical/oral retinoid therapy
• Cosmetic surgery to remove disfiguring scars
• In case of severe symptoms, systemic therapy using antimalarial drugs may be necessary
• Systemic steroid therapy; injection of corticosteroids into the affected region
• In those who limit sun exposure, vitamin D supplements may be administered

Treatment measures for other cutaneous manifestations and underlying systemic lupus erythematosus, if present, may be instituted.

It is important to note that Mucosal Lupus Erythematosus in pregnant women should be carefully evaluated by a qualified healthcare provider before starting and/or performing any treatment. This is because some of the treatment measures may have the potential to affect the developing fetus, and hence, the guidance of a healthcare expert is crucial.

How can Mucosal Lupus Erythematosus be Prevented?

• Currently, there is no effective preventive method available for Mucosal Lupus Erythematosus
• Flare-ups can be prevented by managing medications, avoiding sun exposure, utilizing suitable sunscreens and clothing (cover-up skin exposed to sunlight), and close monitoring of the symptoms
• Avoiding factors that aggravate the condition through the following measures:
  • Avoidance of smoking
  • Undertaking early treatment of viral infections
  • Addressing issues causing hormonal imbalance in the body
• In pregnant women with MLE, careful and periodic monitoring of the pregnancy is advised and recommended
• Educating the individual and keeping them well-informed about lupus is important and beneficial

What is the Prognosis of Mucosal Lupus Erythematosus? (Outcomes/Resolutions)

The prognosis of Mucosal Lupus Erythematosus depends on the severity of the signs and symptoms, its association with systemic lupus erythematosus, and the response to treatment.

• Individuals with mild cases of MLE, who respond well to therapy, have a better prognosis than those with severe symptoms and poor response to treatment
• In many cases, the prognosis is good, and with proper treatment and monitoring of the condition; many individuals are able to lead normal, healthy lives while managing flares
• It is important that appropriate treatment be provided during pregnancy and close monitoring of both the mother and developing fetus be undertaken

Additional and Relevant Useful Information for Mucosal Lupus Erythematosus:

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

https://www.dovemed.com/diseases-conditions/systemic-lupus-erythematosus/