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Medically reviewed by Hugo Benalcazar, Northwestern University on April 14th, 2024.
Key Takeaways
- These conditions include pemphigus vulgaris and bullous pemphigoid, affecting 1-5 people per million annually, with pemphigus carrying higher mortality risk if untreated.
- Blisters form when antibodies attack desmoglein proteins (pemphigus) or basement membrane zone proteins (pemphigoid), disrupting skin cell connections.
- Oral lesions often appear first in pemphigus vulgaris, while bullous pemphigoid typically starts with itchy, tense blisters on the trunk and extremities in patients over 60.
- High-dose prednisone (1-2 mg/kg daily) is standard initial treatment, often combined with steroid-sparing agents like methotrexate or mycophenolate mofetil to reduce long-term steroid complications.
- Rituximab has shown 90% remission rates in severe pemphigus cases, while plasmapheresis can rapidly remove circulating antibodies during life-threatening flares.
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.
For more information on autoimmune blistering disorders, visit:
The Bottom Line
Early recognition and aggressive treatment within the first few months significantly improves outcomes and reduces the risk of life-threatening complications. Distinguishing between different types requires specialized testing including skin biopsy and immunofluorescence studies. If you're experiencing unexplained blistering or oral lesions, Doctronic can help connect you with dermatology specialists for proper evaluation.
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