Understanding Autoimmune Blistering Disorders: Causes, Symptoms, and Treatment Options

Hugo Benalcazar

Medically reviewed by Hugo Benalcazar, Northwestern University on April 14th, 2024.

Autoimmune blistering disorders are a group of rare skin diseases that occur when your immune system mistakenly attacks your skin and mucous membranes, causing painful blisters to form. While there is no cure for these conditions, your doctor can provide treatments to help heal the blisters and prevent complications.

Types of Autoimmune Blistering Disorders

There are several types of autoimmune blistering disorders, including:

  • Pemphigus: Causes soft blisters on the skin and inside the mouth, nose, throat, eyes, and genitals.

  • Pemphigoid: Includes bullous pemphigoid, mucous membrane pemphigoid, and pemphigoid gestationis.

  • IgA mediated bullous dermatoses: Includes dermatitis herpetiformis and linear IgA disease.

  • Epidermolysis bullosa acquisita: Makes the skin fragile, causing blisters from minor injuries.

Symptoms and Complications

The location of blisters depends on the specific disorder. Some cause blisters on the skin, while others affect mucous membranes. Blisters can be painful or itchy and may break open, leaving sores. Complications can include infections, scarring, difficulty eating or breathing, gum disease, tooth loss, and vision problems.

Causes and Triggers

In autoimmune blistering disorders, the immune system mistakenly attacks healthy cells in the skin and mucous membranes. While the exact cause is unknown, certain factors can trigger the condition, such as:

  • Ultraviolet light from the sun

  • Chemicals used to kill pests

  • Hormones

  • Infections

  • Certain medications, such as diuretics, rheumatoid arthritis medications, antipsychotics, and antibiotics

Diagnosis and Treatment

A dermatologist can diagnose autoimmune blistering disorders by examining the skin and performing tests such as a blister biopsy, direct immunofluorescence, or blood tests. Treatment aims to lower the immune system response and prevent it from attacking the skin and mucous membranes. Options include:

  • Corticosteroids to reduce inflammation

  • Immunosuppressive medicines

  • Biologic drugs

  • Intravenous immunoglobulin G (IVIG)

  • Plasmapheresis

Antibiotics, antiviral drugs, or antifungal medications may be prescribed to prevent infections in open blisters.

Home Care and Coping

To prevent infections and promote comfort, keep blisters clean as directed by your doctor. Avoid popping them to prevent scarring. If you have blisters in your mouth, consult your dentist for proper oral care. Each person's experience with autoimmune blistering disorders is unique, so work with your doctor to find the most effective treatment plan for your situation.

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