All About Autoimmune Blistering Disorder

Introduction

Autoimmune blistering disorder is a group of rare skin conditions that occurs when the immune system affects the skin and the mucus membrane, which is a thin lining present in the nose, mouth, etc. This condition results in the formation of blisters. Symptoms caused by this condition are specific in terms of severity, ranging from minimal bumps to lesions that occur throughout the body.

How Does Autoimmune Blistering Disorder Affect the Skin?

The skin, or dermis, is composed of multiple layers that stick together with the help of proteins that act as a protective barrier. Dermis perform many functions that are important to human life, such as regulating the body temperature to protect the blood vessels. The proteins responsible for creating the skin barrier must be healthy and intact. In the case of autoimmune blistering disorder, the autoantibodies attack these proteins, making the epidermis unhealthy.

What Are the Causative Factors of Autoimmune Blistering Disorder?

The exact causative factor of this condition affecting the skin barrier is unknown. Some factors which increase the risk of autoimmune blistering disorder or those that tend to worsen the condition are

• Certain types of food.

• Some medications manage bacterial infections, high blood pressure, and diabetes.

• Ultraviolet light exposure.

• Genetic predisposition.

What Are the Symptoms Associated With Autoimmune Blistering Disorder?

The main symptoms of this condition are the formation of blisters and tension over the skin and mucous membrane. These blisters cause itching, pain, and sores and appear on various body parts depending on the related disease. Other symptoms are:

• Mouth pain.

• Difficult to breathe.

• Difficult to swallow.

• Gastrointestinal bleeding.

• Visual problems.

What Are the Different Types of Autoimmune Blistering Disorders?

The types of autoimmune blistering disorders are:

• Pemphigus Vulgaris: Pemphigus vulgaris is the most common type. This condition is a rare chronic blistering disorder of the skin. This results in the formation of blisters throughout the body and commonly affects the mouth, eyes, throat, nose, and genitals. Usually, blisters cause painful sores on the surface of the skin. However, the blisters caused by this form are not typically itchy. When they burst and erode, they can cause the patient to be more susceptible to infection.

• Pemphigus Foliaceus: The blisters caused by this form usually break away from the dermis quickly and do not affect the mucous membrane. However, they can make the skin scaly and itchy and usually are seen on the scalp, back, chest, and face. They are painless blisters.

• Pemphigus IgA: This form results in the formation of fluid filler blisters, which are generally seen on the arms and legs.

• Paraneoplastic Pemphigus: This form of autoimmune blister disorder is specific to patients with cancer and is characterized by lesions that occur in the mouth and on the lips. This form usually occurs in the sensitive mucous membrane and can even spread to the digestive or respiratory tract. This form of the condition can result in life-threatening situations.

• Bullous Pemphigoid: This form usually affects the older population, which is characterized by reddishness and large blisters. They occur near cuts or wounds and in the armpits, groins, and neck. These blisters are extremely itchy, but heal quickly.

• Cicatricial Pemphigoid: This is a rare form and can result in scarring. When the blisters cause affect both the skin and mucous membrane, it is known as cicatricial pemphigoid, however when it affects only the mucous membrane it is known as mucous membrane pemphigoid.

• Bullous Dermatosis: This form occurs when the immune system attacks the protein present in the subepidermal layer of the skin. This is characterized by cluster formation, which is extremely itchy.

How Are Autoimmune Blister Disorders Diagnosed?

The doctor initially begins by evaluating the physical examination, reviewing the medical and family history, and discussing the symptoms experienced by the person. In order to confirm the type of autoimmune blistering disorder, the doctor may recommend some other tests, which may include:

• Blood Test: A blood test is done to check for the presence of disease-specific autoantibody.

• Skin Biopsy: In a skin biopsy, a small portion of the affected skin is excised and examined under a microscope to see for the signs of disease.

• Direct Immunofluorescence: In this, a small portion of the patient's skin is removed and observed under a special microscope to see for the presence of immune proteins that may be deposited on the patient's skin.

How Is Autoimmune Blister Disorder Managed?

Providing proper treatment for autoimmune blister disorder can eliminate symptoms and prevent complications. There are medications that can suppress the action of the immune system and prevent it from attacking the skin and mucous membranes. These are immunosuppressive drugs, and corticosteroids can be used. The primary medication used to treat this condition is Prednisone, which is a corticosteroid. Additional medications that can be recommended by the doctors are:

• Immunosuppressive biological therapies.

• Antibiotics, antifungal, and antiviral medication to prevent infection.

Autoimmune blister disorders can be managed and treated in most of the cases. Healthy individuals should take prompt action and consult their doctor if they notice anything new or abnormal on the skin.

Home Care: The blisters need to be taken good care of. The person should not pop them, which can leave scars. Some methods need to be followed:

• Wash the sheets, cloth, and towel regularly. Anything and everything that comes in contact with the skin should be clean.

• If the blisters are present in the oral cavity, consult with the dentist to know how to brush the teeth without causing more damage to the blisters.

What Are the Complications Related to Autoimmune Blistering Disorder?

Some common complications are:

• Blisters that open can result in infection.

• They can result in scarring after they heal.

• When they are present in the throat or lungs, they can cause swallowing difficulty.

• In the oral cavity, they can result in gum disease and tooth loss.

• They can affect the eyes.

Conclusion

Autoimmune blister disorder is a rare combination of many skin conditions. This condition is characterized by the formation of blisters or lesions on the skin and mucous membrane which is caused by the immune system attacking the skin. These blisters are painful, itchy, and can cause scars. The symptoms and severity vary in each individual. This condition can be treated with the help of medications and some home remedies.