What is Bullous Systemic Lupus Erythematosus?
Lupus erythematosus is a disease where your immune system mistakenly attacks your own body. It can lead to a wide range of conditions from skin rashes to affecting several organs in the body. Few people with systemic lupus erythematosus, which is a more severe form of Lupus, can develop Bullous systemic lupus erythematosus (BSLE). This condition is quite rare and usually reported through individual cases or small studies. The symptoms of BSLE can be seen as tight blisters on the skin, more often appearing on the limbs, torso, face, and neck. Interestingly, these blisters usually heal without leaving a scar or tiny, white, milky cysts (known as milia).
What Causes Bullous Systemic Lupus Erythematosus?
The exact cause of bullous systemic lupus erythematosus, or BSLE, is not known. BSLE is a rare condition that occurs in people with systemic lupus erythematosus (SLE). It’s related to the body producing antibodies that attack a specific part of the skin, called the dermo-epidermal junction, where type VII collagen is located.
Most people who get BSLE already have been diagnosed with SLE. However, there have been a few instances where BSLE was the first sign that someone had SLE.
Risk Factors and Frequency for Bullous Systemic Lupus Erythematosus
Bullous systemic lupus erythematosus, a specific type of lupus, typically affects more women than men, particularly those of African descent who are in their twenties to forties. However, it can occur in anyone, regardless of their race, age, or gender. Although it’s not very common, it accounts for about 2% of all skin conditions where blisters form because the immune system is attacking the body.
Signs and Symptoms of Bullous Systemic Lupus Erythematosus
Bullous systemic lupus erythematosus (BSLE) often starts suddenly, with tight blisters and skin sores appearing on reddish or normal skin. The blisters tend to show up more on skin exposed to the sun. However, they can also occur on skin not exposed to the sun and the inner lining of the mouth. These blisters commonly appear on the trunk, face, neck, border of the lips, upper arm exterior surfaces, area above the collarbone, and inside of the mouth.
Occasionally, the condition may present with a pattern called ‘erythema gyratum repens’. This rare pattern looks like a red skin sore that expands outward in a circular pattern. It is not common for BSLE to be associated with hives, and itching may or may not be present. Healing can sometimes lead to small bumps, called milia, and/or slight scarring. But what usually happens is that the affected skin changes color, becoming lighter or darker than normal.
Several studies have noted a relation between BSLE and active lupus nephritis, which is a type of kidney inflammation. Out of 128 cases reviewed in one study, 50% had lupus nephritis, usually classified as class III or IV. Also, 7% of the cases had neuropsychiatric SLE (a type of lupus that affects the nervous system), and 45% had blood cell abnormalities at the time of the BSLE diagnosis.
Testing for Bullous Systemic Lupus Erythematosus
Diagnosing the skin condition known as bullous systemic lupus erythematosus requires a careful look at the patient’s symptoms, blood work, tissue analysis, and specific lab tests that use fluorescent antibodies.
Back in 1983, two doctors named Camisa and Sharma suggested a way to diagnose this condition, which was later improved in 1986. According to their criteria:
- You should have been diagnosed with systemic lupus erythematosus (SLE) based on the standards set by the American College of Rheumatology.
- You would have blister-like sores that appear on your skin mainly, but not always, on areas exposed to the sun.
- The tissue analysis of your skin should be similar to what doctors see in a condition called dermatitis herpetiformis, another skin disease that causes itchy blisters.
- Specific lab tests may or may not detect antibodies against a part of the skin called the basement membrane zone (BMZ).
- Another type of lab test must show certain antibodies (IgG, IgM, and often IgA) at the BMZ.
In 1995, another group of doctors defined this condition as a certain type of blister-causing skin disease in a patient with SLE, with immune materials detected at the BMZ.
It’s important to remember that usually, a diagnosis of SLE is present before this skin condition arises. However, in rare cases, this may be the first sign of SLE. Therefore, it’s also important to rule out other diseases that cause blisters on the skin.
Treatment Options for Bullous Systemic Lupus Erythematosus
Dapsone is widely recognized as the primary treatment for certain conditions, often producing great results. However, it’s important to note that dapsone may cause some side effects. These may include conditions such as anemia, liver inflammation, or a severe allergic reaction. If a patient can’t tolerate the drug, shows widespread symptoms, or has considerable skin involvement, steroids may be an alternative.
There are various other agents that can limit the need for steroids, although their effectiveness varies. These medications, which suppress the immune system, include cyclophosphamide, azathioprine, mycophenolate mofetil, and methotrexate. While they’ve shown modest results overall, the drug rituximab has shown potential for cases that don’t respond to other treatments.
On rare occasions, treatments such as anakinra or intravenous immunoglobulins have been utilized and reported in medical literature.
What else can Bullous Systemic Lupus Erythematosus be?
Bullous systemic lupus erythematosus, which is a blistering skin condition, can sometimes be mistaken for other skin diseases that also cause blisters. Dermatitis herpetiformis and epidermolysis bullosa acquisita are two such autoimmune conditions.
Epidermolysis bullosa acquisita, or EBA, also involves antibodies against a protein called type VII collagen, just like bullous systemic lupus erythematosus. However, EBA tends to leave scars or small cysts after healing and lacks other signs of lupus. One distinguishing factor is the absence of a significant characteristic of lupus, large deposits of a substance called mucin in the skin. Additionally, EBA usually responds more slowly to a treatment called dapsone compared to the lupus condition.
Dermatitis herpetiformis, or DH, affects different parts of the skin, typically presenting itchy skin lesions on the outer parts of joints, like the elbows and knees, as well as the buttocks. DH has similar microscopic features to the lupus condition except that it involves deposits of a different type of antibody, called IgA, in the skin.
Other similar conditions include:
- Linear IgA bullous dermatosis: testing reveals IgA deposits on the upper side of a split layer of the skin.
- Systemic lupus erythematosus with blisters: lupus that also causes blisters.
- Bullous pemphigoid: testing finds deposits of antibodies on the upper side of a split skin layer. This condition also leads to skin blisters.
What to expect with Bullous Systemic Lupus Erythematosus
Looking back over 12 years’ worth of data, most patients recovered without any scarring or the appearance of small, white bumps known as milia. However, some patients would sometimes exhibit lighter or darker patches on their skin due to inflammation. The data also indicated that a disease known as BSLE seldom came back.
Possible Complications When Diagnosed with Bullous Systemic Lupus Erythematosus
Complications can arise from various factors, such as:
- Infection of the skin that has been exposed to the disease
- Sloughing esophagitis, which is a severe inflammation of the esophagus
- Complications stemming from a simultaneous flare-up of Systemic Lupus Erythematosus (SLE) affecting multiple organs
- Side effects from the medication used for treatment
Preventing Bullous Systemic Lupus Erythematosus
It’s crucial for patients to learn about their health when dealing with bullous systemic lupus erythematosus, a type of chronic autoimmune condition that can affect multiple body organs. In some people, this disease might be the first indication they have systemic lupus erythematosus (SLE). It’s important for these patients to be aware of the early warning signs that an organ is being impacted by the disease. Furthermore, they should know the importance of protecting their skin from the sun with sunscreen and consistently taking their prescribed medications.
Living with bullous systemic lupus erythematosus can be stressful due to discomfort, appearance concerns, and worries about skin-related complications. Therefore, it may be helpful for these patients to join support groups or consider behavioral therapy. These remedies can provide emotional support and coping strategies for living with the disease.
