Whats The Best Treatment For Bullous Pemphigoid
For mild cases of bullous pemphigoid, the best treatments are topical corticosteroid creams or ointments that you rub directly on the affected areas.
For moderate-to-severe cases of bullous pemphigoid, your healthcare provider may prescribe an oral corticosteroid, like prednisone, in addition to a steroid-sparing immunomodulatory agent such as dapsone, mycophenolate mofetil, azathioprine, methotrexate or chlorambucil.
For refractory cases of bullous pemphigoid, your healthcare provider may prescribe rituximab or IVIG infusions.
What Causes An Autoimmune Rash 10 Possible Conditions
What autoimmune conditions cause a rash on the skin? These are the most common autoimmune diseases that may cause rashes on your skin:
- Lichen planus
- Behçets disease
An autoimmune disease occurs when your bodys immune system attacks healthy cells within the body. This dysfunctional immune response may lead to various symptoms, like hair loss, skin rash, or even joint pain.
More than 20 million Americans suffer from autoimmune diseases such as rheumatoid arthritis or Hashimotos thyroiditis. If you are experiencing unexplained itching, rashes, or skin sores, you should consult your healthcare provider.
Why do autoimmune conditions cause a rash? Autoimmune conditions may cause a rash because they trigger inflammation in skin cells. These diseases are often characterized by chronic inflammation in your internal organs, your skin, and everywhere in between.
What do autoimmune rashes look like? Autoimmune rashes can look like scaly red patches, purplish bumps, or more. The appearance of autoimmune rashes will be different, depending on which autoimmune condition is triggering the skin rash.
For example, cutaneous lupus may cause a scaly red patch that does not hurt or itch. Scalp psoriasis may cause plaque buildup that results in hair loss. Lichen planus may cause purplish, itchy, flat bumps on your skin.
What Causes Bullous Pemphigoid
Bullous pemphigoid is an autoimmune disease. Healthcare providers and medical researchers arent sure why your immune system responds this way.
Certain medications may trigger bullous pemphigoid, including the diabetes medication alogliptin. Ultraviolet light and ionizing radiation may also trigger or aggravate bullous pemphigoid.
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Vitiligo Can Cause Skin To Lighten In Patches
Vitiligo causes your skin to lighten in patches as your body destroys melanocytes, the cells that make pigment. In some cases, your skin may lose all pigment and turn completely white. The condition can affect your hair, the inside of your mouth, and your eyes.
There is no cure for vitiligo, though treatments exist to restore pigment to the skin to even out your skin tone. These include cover-up or foundation, oral and topical medications, and light treatments applied to the affected areas. Light treatments can be time-intensive and costly, and they dont always work. Surgical treatments, such as skin grafting and tattooing, are another option.
What Are The Types Of Pemphigus
There are several types of pemphigus based on where and why lesions develop. Types of pemphigus include:
- Pemphigus vulgaris: This is the most common type in the U.S. Blisters always affect your mouth. Some people may have blisters on their skin and in other mucous membranes. These lesions develop in superficial layers of your skin. They can be painful and heal slowly.
Pemphigus vulgaris causes red and white fluid-filled blisters or open sores to form inside of your mouth.
Pemphigus vulgaris causes blisters to form on your skin. A common location for blisters is near your groin and on the skin on your legs.
- Pemphigus vegetans: This type is similar to pemphigus vulgaris but causes thicker lesions. These lesions usually form in areas of your body with skin folds such as your groin and armpit.
- Drug-induced pemphigus: Medications can cause blistering. Some drugs that cause this condition include antibiotics and blood pressure medication. Blisters can develop months after taking the medicine.
- Pemphigus erythematosus : This type is an overlap syndrome with lupus that causes blisters to develop on your upper back, chest, cheeks and scalp. When lesions form, theyre usually red and scaly.
- Pemphigus foliaceus: This type causes blisters to develop on your scalp, face, neck and back. Lesions rarely appear in your mouth. This type affects your outermost skin layer only. Small blisters may break open easily to form crusty lesions that spread to cover large areas of skin.
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Autoimmune Skin Condition Symptoms
The skin is the largest organ of your body. It serves as a protective barrier to the body against trauma. It also helps with many body functions like regulating your internal body temperature.
While the skin is made up of five distinct layers of skin, and the two top ones are most often affected by autoimmune skin diseases. The top layer is called the epidermis and it is the outermost layer. The underlying layer is the dermis and it contains vital cells, tissues, and structures.
These two layers are held together with proteins and other structures. When there is a separation of the two layers, blisters may form. These blisters can be small or large and contain fluid that contains dead or damaged skin.
Some blisters result from injury to the skin. With autoimmune skin diseases, blisters form because the body has created antibodies that attack proteins required for skin health and function. Sometimes, blisters can break open and become open sores.
In other autoimmune skin diseases, lesions can also form on mucous membranesthe esophagus, throat, inside of the mouth and nasal pathways, the genitals, and anus. Blisters can also cause gastrointestinal bleeding and problems with swallowing and breathing.
Conditions like psoriasis cause an overgrowth of skin cells that pile up on the surface of the skin. These plaques can burn, sting, and itch.
Other symptoms of autoimmune skin diseases include:
Types Of Autoimmune Blistering Disorders
Autoimmune blistering disorders are broken down into different types. These are some of the main ones:
Pemphigoid is another group. It has three main types:
- Bullous pemphigoid usually affects people older than 70. It causes itchy blisters to form on the arms, thighs, and belly.
- Mucous membrane pemphigoid affects the lining of the mouth, eyes, nose, throat, and genitals.
- Pemphigoid gestationis affects women during pregnancy or just after their baby is born. It starts as a bumpy rash on the belly, arms, and legs. Then the bumps turn into blisters.
IgA mediated bullous dermatoses are disorders in which the immune system makes a lot of immunoglobulin A , a type of antibody that fights bacteria, toxins, and viruses. It comes in two types:
- Dermatitis herpetiformis affects people with celiac disease . It causes clusters of itchy blisters to appear on the elbows, knees, scalp, and buttocks.
- Linear IgA disease causes new blisters to form a ring around old ones on the skin. This is sometimes called a âcluster of jewels.â This type affects mucous membranes, too.
Epidermolysis bullosa acquisita mostly affects middle-aged and older adults. It makes the skin so fragile that blisters form from minor injuries.
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What Is Bullous Pemphigoid
Bullous pemphigoid is a rare skin condition that causes itchy, hive-like welts or fluid-filled blisters.
It may affect a small area of your body or it may be widespread. Blisters may occur anywhere, but often develop on flexural areas of your skin, such as under your armpits , around your groin or on your stomach . In some cases, blisters also form on mucous membranes, including your mouth, tongue, throat, esophagus and/or eyes.
Who Is At Risk For Pemphigus Vulgaris
Pemphigus vulgaris isnt contagious and cannot be transmitted from one person to another. It also doesnt appear to be transmitted from parent to child. However, a persons genes can put them at a higher risk for the condition. If your parents or other family members had or have the condition, youre more likely to develop it.
Pemphigus vulgaris can affect people of all races, genders, and ages. However, the condition is more common in the following groups:
- people of Mediterranean descent
How Does Pemphigus Affect My Body
Pemphigus causes blisters and sores to form on your skin. These lesions form quickly and can last for years, with new blisters appearing in the same area of your skin after one blister goes away. These lesions can be painful and cause additional symptoms like infections. Contact your healthcare provider if you have blisters that are widespread across your body, as they could be life-threatening.
Autoimmune Blistering Disorder Causes
When your immune system is working as it should, it attacks bacteria and other invaders before they can make you sick. In autoimmune blistering disorders, your system mistakes healthy cells in your skin and mucous membranes for unwanted intruders.
Your immune system makes proteins called antibodies. These attack the substances that hold the outer and inner layers of skin together. The damage causes the two layers of skin to separate. Fluid collects between the two and forms blisters.
Doctors don’t know what causes the immune system to misfire. Some people have genes that make them more likely to get one of these disorders. Then, something actually triggers it, such as:
- Ultraviolet light from the sun
- Chemicals used to kill pests
Usually, the blisters go away once you stop taking the medicine causing them.
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Autoimmune Blistering Disorder Diagnosis
You’ll see a dermatologist. They will look at your skin and see where the blisters have formed.
You might get one or more of these tests:
- Blister biopsy. Your doctor removes a piece of the blister and looks at it under a microscope. A biopsy can show where the skin layers have separated.
- Direct immunofluorescence. A chemical is placed on the skin biopsy sample. Then it is tested for antibodies. The type of antibodies in your skin can show which type of blistering disorder you have.
- Blood test. Your doctor tests a sample of your blood for antibodies. This test can show how severe your condition is. It can also help the doctor see whether treatment is working.
Sometimes itâs hard to tell the difference between autoimmune blistering disorders and other conditions, including:
- Genetic blistering disease, a group of disorders caused by changes in a gene needed for healthy skin
- Groverâs disease, a rare skin disorder that can cause small red lesions and blisters
- Other skin conditions that cause blisters, such as erythema multiforme or impetigo
Make An Appointment With One Of Our Specialists
A referral is required to meet with an autoimmune skin disease specialist. However, if you have questions about a skin condition without a pre-existing diagnosis, you can schedule an appointment with a general dermatologist at the University of Utah Health clinic by contacting 801-581-2955.
For new patients with an existing diagnosis, you will need a referral from your current provider. Your referral should include your name, date of birth, home address, phone number, and insurance company.
Your autoimmune history records should be mailed or faxed to U of U Health before your first appointment with us . 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
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Can Autoimmune Disorder Cause Blisters
Autoimmune blistering disorders are a group of rare skin diseases. They happen when your immune system attacks your skin and mucous membranes the lining inside your mouth, nose, and other parts of your body. This causes blisters to form.
What autoimmune disease presents with a rash?
Two autoimmune diseases lupus and dermatomyositis have rashes that can affect the face in different ways. Lupus is a chronic autoimmune disease that most commonly affects women age 15 to 44. There are different types of lupus. Some forms mainly affect the skin, like cutaneous lupus.
How do you treat autoimmune blisters?
The mainstay of treatment for autoimmune blistering diseases is treatment with corticosteroids such as prednisone. Corticosteroid therapy is not effective in all cases and long-term treatment with high-doses of corticosteroids can cause serious side effects.
Why am I getting water blisters on my body?
What causes water blisters? When the outer layer of your skin is damaged, your body sends blood to heal and cool the injured area. Part of that process is the formation of protective pads comprised of blood serum . These serum pads are water blisters.
Sun Protection If You Have An Autoimmune Condition
Sun protection is an important part of treatment for many autoimmune conditions, especially lupus and dermatomyositis. Even a small amount of sun exposure can cause skin symptoms to worsen, so its important to wear sunscreen of at least SPF 30 every day.
Psoriasis is one autoimmune condition where ultraviolet light is used as a treatment. While natural sunlight may help with symptoms, its not always recommended. Talk to your provider about whats right for you if you have psoriasis.
Keep in mind that some medications used to treat autoimmune diseases can also make you more sensitive to the sun.
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What Are The Symptoms Of Pemphigus
Symptoms vary based on the type of pemphigus you have but could include:
- Fluid-filled bump or bubble on your skin .
- The skin around the blister is pink to red.
- Sores that have a crusty appearance.
- Blisters or sores leak clear fluid or bleed lightly.
- Your skin around the blisters is fragile and peels in layers or scales.
- Pain on or near your affected skin.
- Itchy skin.
Blisters and sores can easily become infected. Skin symptoms of an infection include:
- White or yellow pus fills the blister and leaks out if the blister breaks open.
- Pain or a burning sensation to the touch.
- Yellow crust forms on the blister if it breaks open.
- Skin doesnt heal.
- The area on or around the blister swells or gets bigger.
Severe symptoms of pemphigus include:
Treatments For Pemphigus Vulgaris
If a GP thinks you could have pemphigus vulgaris, they can refer you to a skin specialist for tests and treatment.
Blisters caused by pemphigus vulgaris usually come and go over time. There’s no cure, but treatment can help.
The main treatments are:
It usually takes a few weeks for the blisters to heal. Once this happens, you’ll probably need treatment for several years to help stop the blisters coming back.
It may be possible to stop treatment eventually. If the blisters come back, you may need to repeat treatment.
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Pathological Features Of Pemphigus
The characteristic histological findings in a biopsy of pemphigus are a split within the epidermis above the basal layer, with epithelial cells floating separately instead of being joined together. The basal cells stand like a row of tombstones on the floor of the blister at the base of the mucosa and epidermis.
A particular test called direct immunofluorescence performed on the biopsy helps to confirm the diagnosis and differentiate pemphigus from other blistering disorders. A positive analysis indicates the presence of antibodies binding to the epithelial cell surface.
The clefting arises because the antibodies bind to proteins called desmogleins, breaking up the plates that stick the keratinocytes together . The keratinocytes separate from each other .
The autoimmune antibodies can also be detected by a skin antibody blood test . It is positive in 80-90% of patients with active pemphigus. Disease activity correlates with antibody titre in most patients.
Expert Diagnosis And Treatment For Autoimmune Blistering Skin Diseases
These blisters tend to be more widespread and heal more slowly than blisters caused by minor burns, friction, or injuries. They also may cause scarring.
Men and women are equally affected by autoimmune blistering diseases. Although most of these disorders occur in people in their 50s and 60s, they can develop in people of all ages, including children.
UT Southwestern is home to a Clinical Laboratory Improvement Amendments -certified Cutaneous Immunopathology Laboratory that is dedicated to the diagnosis and classification of autoimmune skin disorders. Our experienced physicians are recognized leaders in the treatment of both autoimmune diseases and complex skin conditions.
We also offer specialty clinics for patients with autoimmune blistering diseases.
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Sores In The Mouth Or Genitals
Mouth ulcers, like canker sores, are common and usually go away on their own. If mouth ulcers keep coming back, or if you also have them on the genitals, it could be a sign of a rare autoimmune disorder called Behçet disease.
Behçet disease can happen at any age, but it usually affects people in their 20s and 30s. We dont know the exact cause, but many symptoms are caused by inflammation of blood vessels. The condition can be different in each person, and its common for symptoms to come and go. Some other symptoms include:
Pain in the joints
There is no cure for Behçet disease, but symptoms can usually be controlled with different medications. In some people, the symptoms will go away for a period , and treatment may not be needed for a while.
Its important to note that other autoimmune diseases can also cause sores in the mouth or genitals, including lupus and pemphigus. Its a good idea to be checked out by your provider to determine what the underlying cause is.