People with autoimmune diseases often face a complicated journey to diagnosis and treatment. Those whose skin is affected face the additional stigma of having symptoms that are visible to everyone they meet.
Doctors in Yale Medicine's dermatology and rheumatology departments work closely together to help people with autoimmune diseases such as lupus and scleroderma look and feel as good as possible. They not only provide warm, personal patient care, but also participate in research to advance treatment of skin conditions associated with autoimmune diseases for people all around the world.
Autoimmune diseases tend to bring complicated symptoms. Many people with these conditions see doctors in several medical specialties. Lupus and scleroderma are two that primarily affect the skin, requiring dermatology care. But these diseases may also affect connective tissues, which are treated by a rheumatologist. “We aim for an interdisciplinary approach for patients who may require the expertise of multiple specialists,” says Yale Medicine dermatologist Sarika Manoj Ramachandran, MD.
What are some of the autoimmune diseases that affect the skin?
When a person’s immune system starts fighting against the cells it’s supposed to protect, it can lead to autoimmune diseases, several of which affect the skin and internal organs. In most of these skin-involved diseases, symptoms can vary greatly from person to person. (For example, not all patients with one of these autoimmune diseases will eventually have symptoms that affect internal organs.) Two of the most common such conditions are lupus and scleroderma.
- Lupus: A disease called lupus leads to a wide variety of symptoms, many of which can resemble other skin diseases. When it affects the skin, the condition is known as cutaneous lupus (or skin lupus). It can come in many form —most commonly, patients will see a butterfly-shaped rash, often on the face. When it spreads beyond the skin, it’s known as systemic lupus erythematosus (SLE), and the inflammation can affect the joints, the kidneys and other organs.
- Scleroderma: Like lupus, scleroderma can affect the skin, or it can become systemic. In this case, the immune system produces too much collagen, causing the skin to tighten and become tough and hard. When scleroderma is localized, or mainly affecting the skin, it can either be morphea, which manifests as oval patches, or linear, which shows up as streaks of hard patches. When scleroderma is systemic, it can develop slowly or quickly, affecting internal organs such as the lungs, heart and kidneys.
What causes these autoimmune diseases?
The causes for autoimmune diseases that affect the skin are still largely unknown, though doctors continue to research in order to help better understand—and treat—them.
Whilelupus, for example, is known to be set off by sun exposure and specific stressors in some people, the underlying causes of the disease are still under investigation.Dr. Ramachandran says that some medications, including some anti-hypertensives and anti-fungals, have been implicated in the onset of lupus. In those cases, symptoms generally go away after the drug is no longer used.
“Although we don’t know exactly why certain people get lupus,” Dr. Ramachandran says, “we do think there can be both an environmental trigger and then underlying tendencies toward developing it, and those, in conjunction, may bring it out in people.”
The underlying causes of scleroderma are also unknown. Like lupus, some environmental factors may play a role, including some industrial chemical toxins such as epoxy resins and silica. Also like lupus, genetics may play a role in causing the disease.
Lupus is more likely to develop in women in their 20s and 30s, though it can develop in patients of any sex, age, race or ethnicity. Similarly, scleroderma, which is somewhat rare, commonly affects women, and generally affects adults in their mids to 50s. Still, like lupus, it can appear in every age group and across every sex, race and ethnicity.
How are these autoimmune diseases diagnosed?
Because conditions such as lupus and scleroderma have symptoms that can resemble many other diseases, they can be difficult to diagnose initially. Lupus, for instance, can have symptoms that include fatigue and fever. Symptoms of scleroderma can include heartburn.
When you notice the first symptoms—generally some form of skin rash that doesn’t go away or that worsens over time—you may seek treatment from a primary care doctor or dermatologist. An examination with a doctor can include discussion of symptoms, personal medical history and family history, as well as blood and other laboratory tests (including possibly a skin biopsy, in certain cases of potential scleroderma). The patient may then consult with a rheumatologist, who will have more experience working with the internal symptoms of the disease. Over time and depending on his or her symptoms, a patient may work with either a dermatologist or a rheumatologist, or both.
How are these autoimmune diseases treated?
While there are currently no curative treatments for these autoimmune diseases, there are courses of action that can lead to periods of remission or a reduction in flare-ups. Depending on the symptoms, there is a range of options that may be used singularly or in conjunction with each other. The symptoms that affect the skin are often the first and sometimes the only ones that appear and are often comparatively easier to treat than those that affect internal organs.
- Lupus: Over-the-counter anti-inflammatory drugs such as Motrin (ibuprofen) may be used in some patients to treat swelling and pain. Another option to treat inflammation is corticosteroids, though side effects can include a higher risk of contracting infections. Anti-malarial drugs including Plaquenil (hydroxychloroquine) are often used to treat the symptoms of lupus because they’ve been shown to alleviate joint pain, improve skin rashes and help reduce flare-ups significantly. Doctors may also prescribe immunosuppressants such as Azasan (azathioprine), which suppress the immune system to prevent it from attacking the cells it shouldn’t be attacking.
Additionally, because ultraviolet light can cause flare-ups, patients are advised to be very careful with sun exposure, wearing sun-protective clothing, hats and sunscreen with a high SPF. “There are certain local treatments that can be helpful but number one is sun protection,” says Dr. Ramachandran.
- Scleroderma. Because vascular disease can commonly occur in patients with scleroderma, drugs such as some blood pressure medications may be used to open up the blood vessels. As with lupus, over-the-counter anti-inflammatory drugs, corticosteroids and immunosuppressants can also help to reduce symptoms of scleroderma. “A lot of these autoimmune conditions can be treated with immunosuppressant medications,” Dr. Ramachandran says. “But while you’re on them, you need to be closely monitored by your physician to make sure you’re not getting infections.”
What makes Yale Medicine’s approach to treating these autoimmune diseases unique?
“At Yale Medicine, we get together to discuss complicated cases to try to give the patients an interdisciplinary approach to their care,” says Dr. Ramachandran. “There’s definitely an advantage to have everyone on the same page," she says.
Yale Medicine dermatologists also receive many referrals from doctors throughout the region.
"Through referrals, we see some of the more complicated cases,” Dr. Ramachandran says. “The doctors here truly care about their patients and try to come up with the best possible treatment regimens, working together to help patients with more challenging conditions.”
Rheumatic skin disease: Frequently asked questions
What is autoimmunity?
Almost all of the diseases in this category are thought to be diseases in which the immune system is not working properly. The immune system is a normal part of the body that is designed to protect us from infections with germs and viruses from the environment. Our immune system also helps us ward off cancer cell development in our bodies.
When the immune system gets out of control and starts attacking our own bodily tissues, it is called autoimmunity. In this situation, blood proteins called autoantibodies are produced that bind to and injure our own bodily tissues. Rheumatic diseases such as rheumatoid arthritis, lupus, dermatomyositis, and scleroderma are thought to be autoimmune diseases.
What are the typical skin symptoms of autoimmune diseases?
Subacute cutaneous lupus erythematosus (SCLE)
Subacute cutaneous lupus erythematosus (SCLE) is a form of lupus skin disease that is made worse by exposure to sunlight or artificial sources of ultraviolet radiation and does not produce scarring. It produces scaly red patches on the skin that can simulate the appearance of psoriasis occurring in sun-exposed areas of the body.
Patients with this form of skin lupus have a somewhat higher risk for developing the more severe internal complications of systemic lupus erythematosus compared to another common form of lupus skin disease named discoid lupus erythematosus.
Discoid lupus erythematosus (DLE)
Discoid lupus erythematosus (also referred to as DLE) produces scaly coin-shaped lesions most commonly occurring on the face or scalp, although other parts of the body can be affected. This type of skin lupus often produces scarring of the skin and hair loss that can be permanent. In addition, discoid lupus skin lesions often produce darkening and/or lightening of the skin color. When lupus shows itself initially only as discoid lupus skin lesions, such patients are at very low risk for later developing serious internal problems from systemic lupus.
Neonatal lupus is a condition in which newborn babies develop skin lesions often simulating the appearance of subacute cutaneous lupus erythematosus. However, this occurs only when the mother of the newborn baby also had an immunological abnormality during pregnancy that resulted in her body producing Ro autoantibodies. It is thought that the mother's Ro autoantibodies cross over into the baby's blood circulation while still in the womb. These autoantibodies appear to be actually causing the skin lesions that occur after the baby is born and exposed to sunlight and other forms of ultraviolet light.
Complications of neonatal lupus
Normally neonatal lupus erythematosus (NLE) is a mild condition and the skin lesions go away on their own as the child gets older when the autoantibodies from the mother's blood disappear from the baby's blood. However, there is another complication that can occur in this setting and that is congenital heart block. Rather than developing skin lesions after delivery, babies develop damage in the conduction system in their hearts while still in the womb. This can be a very severe complication requiring permanent pacemaker placement in the heart of the baby.
The third condition I mentioned that is associated with Ro antibodies is Sjogren's syndrome. This is a condition that produces dryness in the eyes and mouth, most commonly in adult women. This dryness results from autoimmune damage to the glands that make tears and saliva. This is one of the most common of all rheumatic or arthritis-associated diseases but among the most difficult to diagnose–it is very often not diagnosed until it is quite advanced.
Patients who have Sjogren's syndrome also experience body tenderness and lethargy that can simulate conditions such as fibromyalgia. In addition, internal organ damage can occur in Sjogren's, such as kidney problems, nerve injury, and blood vessel injury.
What are the blood tests for autoimmune diseases?
There are some blood tests one can do that can reflect how active the immunological disease is inside our bodies in diseases like rheumatoid arthritis, lupus, and scleroderma. These tests are routinely ordered by doctors who are treating such patients. This information can be very helpful at times both in the diagnosis of the specific problem and in guiding treatment of the problems over time.
In a disease like rheumatoid arthritis, the most common blood test abnormalities are the presence of rheumatoid factor. Rheumatoid factor is a type of autoantibody present in the blood of almost all patients with rheumatoid arthritis who have the really destructive form of the disease.
This particular test also indicates a risk for some of the complications that can occur in rheumatoid arthritis, in parts of the body outside of the joints. A test like the rheumatoid factor certainly can be helpful in making the initial diagnosis of rheumatoid arthritis. In addition, the amount of rheumatoid factor in the blood can be an indication of the state of activity of the immunological illness inside the body.
Antinuclear antibody assay (ANA)
Another test is the antinuclear antibody assay (the "ANA test" for short). The ANA test is almost always positive in rheumatic diseases such as lupus. In addition, it is often positive in rheumatoid arthritis, dermatomyositis, and scleroderma.
This test, since it can be positive in a number of these diseases, is not diagnostic of any one particular disease. A physician will use the ANA test to screen for this general group of illnesses, and if that is positive, then will do more specific tests to make a specific diagnosis such as lupus.
However, one thing that must be kept in mind in interpreting the results of the ANA test is that it can also be positive in other disease settings that are not related to arthritis. Even normal individuals, on occasion, will have abnormal ANA test results. This occurs even more frequently in older, healthy individuals. Certain medications can trigger a positive ANA test. The point is that the physician must be very careful in interpreting the results of the ANA test and should counsel patients about the true meaning of an ANA test result. I see a lot of confusion produced as a result of physicians in the community not being fully aware of the various pluses and minuses of the ANA test.
Antibody to neutrophil cytoplasmic antigens (ANCA)
I will mention one other laboratory test in this context. That is the ANCA test. ANCA stands for "antibody to neutrophil cytoplasmic antigens." This test is often positive in forms of blood vessel inflammation such as vasculitis.
One of the strongest disease associations of the ANCA test is a disease called Wegener's granulomatosis. This is a disease that can attack blood vessels in different parts of the body, including the skin. Recognizing the characteristic patterns of skin changes can be a clue to the diagnosis of this disease and getting patients on proper treatment for the internal complications that can be very severe (lung and kidney injury).
However, like the ANA test, one must be careful in interpreting the ANCA test results. The ANCA test can be positive in other conditions besides vasculitic illnesses, such as Wegener's granulomatosis.
The treatment really has to be individualized to the specific disease and to the specific conditions related to a given case. Some drugs might be riskier in women compared to men, for example.
How are rheumatic skin conditions treated?
The answer to this question is complex. The skin lesions in a large number of the or so diseases that cause arthritis are treated differently. For example, in a disease like lupus, the skin lesions can be treated quite nicely with cortisone-containing creams and oral medications such as the antimalarials. However, these same forms of treatment usually do not help the skin changes that we see in scleroderma.
The treatment really has to be individualized to the specific disease and to the specific conditions related to a given case. Some drugs might be riskier in women compared to men, for example.
Some drugs like the corticosteroids ("steroids") suppress the immune response in a broad fashion and can be very useful in a number of autoimmune diseases, including the rheumatic diseases (corticosteroids are commonly referred to as "cortisone" type drugs). Corticosteroids like prednisone taken by mouth can certainly suppress various manifestations of rheumatoid arthritis, including the skin changes like vasculitis.
However, long-term use of corticosteroids by mouth can produce a lot of troublesome and serious side effects. Therefore, physicians are constantly trying to find other drugs that will prevent having to rely on corticosteroids for long periods of time.
Methotrexate instead of corticosteroid
In the case of rheumatoid arthritis, methotrexate is a drug that has been found to be able to prevent patients from having to take so much corticosteroid. Generally, the things a dermatologist does to treat the surface of the skin, such as applying sunscreens and corticosteroid-containing creams or ointments, does not help the more severe skin problems such as vasculitis that are seen in rheumatoid arthritis.
What are treatment options for lupus skin disease?
Regarding treatment of lupus skin disease, the topical measures that were discussed above, such as the application of sunscreens and corticosteroid-containing creams and ointments directly to the skin, can be helpful in suppressing the skin inflammation caused by lupus.
Medications for lupus skin diseases
However, most skin lupus patients do require some type of oral therapy in addition to the topical therapy. The safest form of oral therapy to treat lupus skin disease would be one, or a combination, of the anti-malarial drugs, such as hydroxychloroquine, which is commonly referred to by its trade name: Plaquenil. This drug can be used very safely if the common recommended guidelines concerning total daily dosage are followed. However, patients need to have their eyes monitored while on this drug since on rare occasion problems can develop in the retina of the eye while on this form of treatment.
How are skin problems from dermatomyositis treated?
Similar forms of treatment are used for the skin problems seen in patients with dermatomyositis and lupus. Dermatomyositis causes autoimmune inflammation and damage in the muscles, skin, and occasionally other vital organs, such as the lungs.
However, dermatomyositis skin disease generally is harder to treat than is lupus skin disease. In addition, dermatomyositis skin disease is often more troublesome for the patient by producing symptoms such as itching (lupus skin disease usually does not itch).
How does scleroderma affect the skin?
Scleroderma is a term that just means 'hard skin.' Like lupus, patients having scleroderma skin changes have a variable risk for having associated damage to internal organs, especially the kidneys and lungs.
Some patients develop a form of scleroderma that never goes on to cause damage to internal organs. This form of the disease is called localized scleroderma or morphea. However, other patients with scleroderma do develop internal complications relatively soon after the onset of skin problems.
A visual guide to 6 conditions that cause skin discoloration
Learn the difference between vitiligo and conditions like scleroderma or Addison disease
Vitiligo is not the only disease that causes skin to change color.
Here are a few conditions and what they look like, including vitiligo.
Is it Vitiligo or something else?
Vitiligo is an autoimmune disease that causes your skin to lose color, often resulting in white patches on your skin that cover both sides of your body.
Pityriasis alba is a common skin disorder that causes areas of light-colored skin. The spots often start as slightly red, scaly patches on the face, upper arms, neck, and upper middle section of the body.
Tinea versicolor is caused by a fungal infection and leads to patches of discolored skin with a fine, dry, scaly surface.
Hansen's disease (leprosy) is a bacterial infection that can also cause discolored patches of skin. Usually these patches are flat, look faded, and may even feel numb.
Scleroderma is a rare but serious autoimmune disease that causes parts of the skin to harden. Often those areas can appear shiny. It can also create problems with connective tissue beneath the skin, harming blood vessels and internal organs.
Addison disease happens when the body doesn't produce enough cortisol, a hormone that regulates stress. One symptom of the disease is darkening of the skin in places like scars, skin folds, elbows, knees, and knuckles.
Talk to your health care provider if you think you might have vitiligo or another skin condition.
Image credit: Adobe Stock
An autoimmune disease is a disorder in which the body is attacking itself. Normally, white blood cells produce antibodies that attack harmful cells as they appear in the body. The opposite happens in autoimmune diseases. Antibodies attack healthy tissues instead of the harmful ones.
This causes many different symptoms that affect the joints, internal organs, and skin.
Autoimmune diseases can affect many parts of your bodyincluding your skin. Because its so visible, you can often see symptoms of autoimmune disease first on the skin.
These autoimmune skin diseases can show in a variety of ways. Symptoms can include:
- fatigue, and
- scaly patches.
Types of Autoimmune Skin Diseases
Autoimmune skin diseases cannot be cured, but we can help manage flare ups with treatment. Dermatology services at University of Utah Health offers a specialty autoimmune clinic designed to evaluate and treat patients with autoimmune conditions that affect their skin.
Our patients are treated by multiple board-certified dermatologists who specialize in autoimmune skin diseases. Our dermatologists give each patient extensive evaluation and treatment.
Healthy skin will only form a blister after your skin becomes damaged or dead. In skin suffering from an autoimmune blistering disease, your bodys immune system mistakes normal skin tissue for something it needs to fight off, and then attacks this healthy skin tissue. This causes blisters to form.
Several autoimmune skin diseases are responsible for causing skin blistering, including:
- Epidermolysis bullosa acquisita
- IgA-mediated bullous dermatoses
- Ocular cicatrical pemphigoid
If you are a new patient, youwill need a referral from your current provider. Your referral should include your demographic information, includingyour name, date of birth, home address, phone number, and insurance company.
Records about your autoimmune history should be mailed or faxed to University of Utah Health before your first appointment with us (see address and fax number below). These records should include clinic notes, biopsy reports, lab reports, diagnostic studies, radiographic studies, and treatments.
Please make sure your doctor sends your referral to:
University of Utah
Department of Dermatology
Attn: Autoimmune Clinic
Phone:, ask for autoimmune scheduling
We will review referral requests within 48 hours to make sure that the best dermatologist evaluates each case. We will contact new patients about an appointment.
Discoloration autoimmune disease skin
Autoimmune Skin Disease & Disorders
Autoimmune diseases can cause the body to react in many different ways. An autoimmune disease causes a person's immune system to attack the body's own tissues and organs. Because the skin is the body's largest organ, it is commonly affected by certain autoimmune disorders.
At University Hospitals, our dermatology team is experienced in diagnosing and treating skin-related autoimmune disorders and diseases. We use a variety of both topical and oral prescription medications, along with other therapies, to help each patient achieve and maintain clear skin.
Conditions we treat include:
- Blistering diseases
- Bullous pemphigoid
- Epidermolysis bullosa
Common Autoimmune Skin Disorders and Treatments
- Bullous pemphigoid, pemphigus, and epidermolysis bullosa aquisita: These rare disorders cause large fluid-filled blisters to develop on the skin. Treatments may include corticosteroids, immuno-suppressing medications, and anti-inflammatory drugs.
- Dermatomyositis: This conditions can cause a distinct skin rash, discoloration and swelling. It can usually be treated with steroids and other medications.
- Scleroderma: This disorder can cause skin thickening, scarring and inflammation, and can be treated with prescription moisturizers or corticosteroid creams.
- Vasculitis: This condition causes inflammation of the blood vessels, and can affect many parts of the body, including the skin. Treatments include steroids and other medications.
Our Specialty Immuno-Dermatology Clinic treats autoimmune skin diseases such as bullous dermatosis, atopic dermatitis, vasculitis and lupus using evidence-based immunotherapies.
Features of our program include immunofluorescence lab facilities, immune-mapping of congenital blistering disorders, and subspecialty expertise in complex disorders of the skin.
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