Autoimmune Blistering Skin Diseases

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UT Southwestern Medical Center delivers advanced, specialized care to patients with autoimmune blistering skin diseases. Our experts provide diagnostic and therapeutic interventions that optimize the management of these rare, potentially complicated skin diseases.

Expert Diagnosis and Treatment for Autoimmune Blistering Skin Diseases

Autoimmune blistering skin diseases are rare conditions in which the immune system mistakenly attacks proteins necessary for skin layers to stick together. This causes painful blisters that affect the skin and mucous membranes.

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 (CLIA)-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.

Types of Autoimmune Blistering Skin Diseases

Autoimmune blistering skin diseases include:

  • Pemphigus: A group of intraepidermal blistering disorders that cause erosions and/or sores on the skin, mouth, throat, nose, eyes, scalp, or genitals (includes pemphigus vulgaris and pemphigus foliaceous)
  • Pemphigoid: A group of subepidermal blistering disorders that cause vesicles, blisters, erosions, and/or sores on various areas of skin and mucous membranes (includes bullous pemphigoid, mucous membrane pemphigoid, pemphigoid gestationis, and other disorders)
  • IgA-mediated bullous dermatoses: A group of subepidermal blistering disorders that cause blisters of skin and/or mucous membranes (includes dermatitis herpetiformis and linear IgA dermatosis)
  • Epidermolysis bullosa acquista: A rare subepidermal blistering disease that causes fragility and/or blisters of the skin and mucous membranes

Causes and Risk Factors of Autoimmune Blistering Skin Diseases

Doctors aren’t sure what causes the immune system to mistake the skin and mucous membranes for harmful targets and attack them. Certain factors can increase the risk of autoimmune blistering skin diseases or make them worse, such as:

  • Certain foods
  • Certain medications, such as those for high blood pressure, diabetes mellitus, and bacterial infections
  • Exposure to ultraviolet light
  • Genetic predisposition

Symptoms of Autoimmune Blistering Skin Diseases

The main symptom of autoimmune blistering diseases is blisters or lesions on the skin or mucous membranes that can cause itching, pain, or sores. The blisters or lesions appear in different areas of the body, depending on the associated disease.

Other symptoms occur more rarely and include:

  • Mouth pain
  • Difficulty breathing
  • Difficulty swallowing
  • Gastrointestinal (digestive tract) bleeding
  • Visual difficulties

Diagnosing Autoimmune Blistering Skin Diseases

Our skilled dermatologists have years of expertise evaluating symptoms and diagnosing autoimmune skin diseases. We begin with a complete evaluation, which includes a:

  • Physical exam
  • Review of personal and family medical history
  • Discussion of symptoms

To confirm a diagnosis of a specific autoimmune blistering skin disease, we typically order further testing. Tests that patients might need include:

  • Blood tests: Drawing of blood to check for the presence of a disease-specific autoantibody (for example, an immune protein that targets skin and causes the blistering disease)
  • Skin biopsy: Removal of a small sample of affected skin for examination under a microscope for signs of disease
  • Direct immunofluorescence: Removal of a small sample of normal skin for examination under a special microscope to allow detection of immune proteins deposited in patient skin

Treating Autoimmune Blistering Skin Diseases

Treatment of autoimmune blistering diseases can relieve symptoms and prevent complications. Medications can reduce the immune system response and prevent attacks on the skin and mucous membranes.

The primary treatment for these disorders is treatment with corticosteroids such as prednisone. Additional medications that our doctors might prescribe include:

  • Immunosuppressive biological therapies such as rituximab
  • Immunosuppressive drugs, such as azathioprine, cyclophosphamide, or mycophenolate
  • Antibiotic, antifungal, or antiviral medications to prevent infections

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