Introduction
Autoimmune diseases occur when the body's natural defense system fails to distinguish between its own and foreign cells, and the body mistakenly attacks normal cells. There are around 80 autoimmune diseases that affect different parts of the body. Research on autoimmunity suggests that these diseases are likely due to the interplay of genetic and environmental factors. Gender, race, and ethnicity are also associated with the likelihood of developing autoimmune diseases. In addition, autoimmune diseases occur more frequently when people encounter certain environmental influences.
What Is Bullous Autoimmune Dermatoses?
Bullous autoimmune dermatoses are a group of rare skin conditions that cause itching, hive-like fluid accumulation, or fluid-filled blisters on a person’s skin. It can affect a small area of the body, or it can be extensive. Blisters can appear anywhere but often occur on skin bends, such as in the armpits, around the groin, or on the stomach. In addition, blisters occasionally form on mucous membranes such as the mouth, tongue, throat, esophagus, and eyes. Bullous autoimmune dermatoses are autoimmune conditions. This means that bullous autoimmune dermatoses occur when the body's immune system attacks the layers of tissue beneath the top layer of skin. Bullous autoimmune dermatoses consist of the following conditions:
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Pemphigus Vulgaris - This condition causes blisters on the skin and mucous membranes all over the body.
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Paraneoplastic Pemphigus - This condition is associated with malignancy.
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Bullous Pemphigoid - It is a rare skin disease primarily affecting older people. It usually begins with an itchy, raised rash. As the disease progresses, large blisters develop on the skin.
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Cicatricial Pemphigoid - It is a rare, chronic, blistering, autoimmune disease that can lead to scarring. It may affect the skin, mucous membranes, or both skin and mucous membranes. Therefore, the disease is often called mucous membrane pemphigoid when only mucous membranes are affected.
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Dermatitis Herpetiformis - Dermatitis herpetiformis (DH) is a chronic autoimmune blistering skin condition characterized by intensely itchy blisters filled with a watery fluid. This may be associated with a gluten allergy or celiac disease.
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Linear Immunoglobulin A Disease - An acquired autoimmune blistering disease can present with a clinical pattern of blisters indistinguishable from dermatitis.
What Are the Causes of Bullous Autoimmune Dermatoses?
Autoantibodies are antibodies made against substances formed by an individual's own body. Autoantibodies can directly destroy cells that carry substances or make them easier for other white blood cells (immunity cells of the body) to destroy. Some autoimmune diseases are caused by autoantibodies. For example, bullous autoimmune dermatoses are a group of blistering diseases. Autoimmune diseases originate when the body's natural defenses against foreign or invading organisms attack healthy tissue for unknown reasons. Autoantibodies bind to components of the skin that hold the dermis and epidermis together, causing these two layers to separate, creating a blister. These proteins are part of a hemidesmosome complex that holds the skin together and provides the skin's structural support. The skin becomes more fragile when the body attacks these proteins, revealing clinical symptoms of bullous autoimmune dermatoses.
What Are the Risk Factors of Bullous Autoimmune Dermatoses?
The major risk factors which increase the risk of getting bullous autoimmune dermatoses are listed below:
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Medication.
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Genetics.
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Smoking.
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Already have an existing autoimmune condition.
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Exposure to toxins.
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Females are more susceptible to getting an autoimmune condition.
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Infection.
The risk of getting bullous autoimmune dermatoses increases with the following conditions:
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Parkinson’s disease (a disease that affects the nerves leading to tremors).
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Dementia (a condition that affects memory).
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Stroke (blocked blood supply of the brain).
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Multiple sclerosis (destruction of the protective covering of the nerves).
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Epilepsy (seizure disorder).
What Are the Symptoms of Bullous Autoimmune Dermatoses?
The signs and symptoms of bullous autoimmune dermatoses are listed below:
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Bullous autoimmune dermatoses present as itchy welts that look like hives or multiple itchy blisters (blisters).
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It often appears in the arms, legs, abdomen, and mouth.
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Blisters usually appear along skin folds like groins and armpits.
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They are usually painless but may rupture and form painful sores or ulcers.
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The fluid in the blister may be clear or contain blood.
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The skin around the blister may appear normal in color or discolored (purple, red, brown, or slightly darker than normal skin color).
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Welts and blisters rarely lead to scarring.
Other symptoms that may accompany:
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Rashes.
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Dry eyes.
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Itching.
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Dry mouth.
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Hair loss.
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Dry skin.
How Are Bullous Autoimmune Dermatoses Diagnosed?
The doctors may recommend the following tests to confirm the diagnosis:
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Check for Nikolsky Sign - The superficial layer of skin slips on slight rubbing, then the sign is positive.
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Skin Biopsy - The doctor will take a small skin sample and send it to a laboratory.
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Blood Test - The doctor will take the blood sample from the vein using a small needle. It is then sent to the laboratory for testing.
What Is the Treatment of Bullous Autoimmune Dermatoses?
In mild cases of bullous autoimmune dermatoses, the best treatment is to rub a topical corticosteroid cream or ointment directly onto the affected area. For moderate to severe cases of bullous pemphigoid, physicians may prescribe oral corticosteroids such as Prednisolone in addition to steroid-sparing immunomodulators such as Dapsone, Mycophenolate mofetil, Azathioprine, Methotrexate, or Chlorambucil may be prescribed by the doctor. To avoid infection in open wounds, Tetracycline or Doxycycline tablets are prescribed by doctors. For refractory cases of bullous pemphigoid, doctors may prescribe Rituximab.
What Are the Complications of Bullous Autoimmune Dermatoses?
The most common complication of bullous autoimmune dermatoses is skin infections. If left untreated, blisters and rough patches can cause discomfort. Rough patches carry the risk of serious infection. Bullous autoimmune dermatoses usually last one to five years and then often diminish or disappear.
How to Manage Bullous Autoimmune Dermatoses?
The tips listed below can help manage the condition.
For Blisters on the Skin:
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Wear soft, loose-fitting clothing made from natural fibers.
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Avoid too much exposure to the sun and wear sunscreen.
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First, wash wounds and sores with antibacterial soap and water to prevent infection.
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Next, apply an antibiotic ointment to the affected area and wrap it in a non-adhesive (non-stick) bandage.
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In the case of bullous pemphigoid on the feet, do not stand or walk for long periods. Instead, moisturize the skin with lotions, creams, coconut oil, or shea oil.
For Blisters in the Mouth:
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Eat soft foods such as soups, purees, puddings, and applesauce.
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Wait for hot food to cool before eating.
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Avoid chewy or spicy foods such as potato chips, cereals, crispy bread, and raw vegetables and fruits.
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Avoid acidic foods such as pepperoni, salsa, citrus fruits, and tomatoes. Avoid drinking alcohol.
Conclusion
Autoimmune conditions are caused because of immune cells of the body attacking the body itself. Bullous autoimmune dermatoses are a group of conditions that affect the skin leading to blister formation. These blisters can burst, leading to open sore wounds. People with bullous autoimmune dermatoses make antibodies against the membrane between the top layer of skin (epidermis) and the next layer (dermis). This antibody attack causes fluid to collect as blisters between these two skin layers. These types of conditions are treated with the application of corticosteroids and immunosuppressants. The condition often improves with the medication over a while. However, since it is a recurrent condition, it may reappear again.
