Bullous Pemphigoid: Symptoms, Causes & Treatment

Bullous pemphigoid is an autoimmune skin condition that causes large, fluid-filled or blood-filled blisters to appear on the skin. These blisters are usually skin-colored or reddish and are often accompanied by intense itching.

Bullous pemphigoid occurs most often in older adults but can occasionally appear in newborns. In some cases, certain medications can trigger the condition, meaning it may also affect younger individuals.

Treatment for bullous pemphigoid should be directed by a dermatologist and started as early as possible. Management usually involves medications such as corticosteroids, immunosuppressants, or monoclonal antibodies to reduce inflammation and suppress the overactive immune response.

Main symptoms

The main symptoms of bullous pemphigoid include:

• Large blisters on the skin;

• Blisters filled with clear fluid or blood;

• Severe itching;

• Wounds, ulcers, and crusting when blisters rupture.

Symptoms of bullous pemphigoid most often develop in skin folds, such as the groin, elbows, and knees. Although less common, blisters may also appear on the abdomen, feet, or in the oral and genital regions.

Blisters typically develop weeks or months after the appearance of firm, raised skin lesions called papules, which can cause moderate to severe itching.

If symptoms of bullous pemphigoid appear, a dermatologist should be consulted for diagnosis and appropriate treatment.

Confirming a diagnosis

Bullous pemphigoid is diagnosed by a dermatologist based on the patient’s symptoms, medical history, age, and a physical examination of the affected skin.

According to the MSD Manual Professional Edition, diagnosis is typically confirmed with a skin biopsy and immunofluorescence testing. This biopsy helps distinguish bullous pemphigoid from other blistering or inflammatory skin conditions with similar symptoms, such as pemphigus vulgaris, scabies, contact dermatitis, or Stevens-Johnson syndrome.

Pemphigus vs pemphigoid

Both pemphigus and bullous pemphigoid are autoimmune diseases that cause blistering of the skin. However, the characteristics of the blisters are different.

In pemphigus, the blisters are fragile and rupture easily, leading to superficial wounds or ulcers. They may also cause a burning or stinging sensation on the skin or mucous membranes.

In bullous pemphigoid, the blisters are firmer and more tense, meaning they are less likely to rupture. They also tend to cause intense itching.

Possible causes

Bullous pemphigoid is an autoimmune disease in which antibodies mistakenly attack the proteins that anchor the epidermis to the underlying dermis, treating these proteins as foreign substances. For this reason, the condition most often occurs in older adults.

In some cases, bullous pemphigoid can be triggered by certain medications, including furosemide, spironolactone, amoxicillin, nonsteroidal anti-inflammatory drugs (NSAIDs), gliptins, and some monoclonal antibodies. When drug-induced, symptoms typically develop within about three months of starting the medication and are more likely to occur in younger individuals.

Is bullous pemphigoid contagious?

Bullous pemphigoid is not contagious and cannot be transmitted from one person to another.

Treatment options

Treatment for bullous pemphigoid should be supervised by a dermatologist or primary care provider. The goal of treatment is to relieve symptoms, prevent disease progression, and improve quality of life.

The main treatment options for bullous pemphigoid include:

1. Corticosteroid ointments

Topical corticosteroid ointments may be recommended when bullous pemphigoid affects less than 20% of the body surface, particularly in older adults.

Potent corticosteroids such as clobetasol propionate are commonly prescribed.

In some cases, the doctor may combine corticosteroid ointments with other medications, such as nicotinamide, tetracycline, minocycline, or doxycycline.

2. Oral corticosteroids

Oral corticosteroids in tablet form may be recommended when bullous pemphigoid affects larger areas of the skin.

In these cases, prednisone or prednisolone are the most commonly prescribed medications.

3. Immunosuppressants

Immunosuppressants may be recommended if oral corticosteroids are not effective in controlling symptoms or if the patient cannot take corticosteroids.

These medications reduce immune system activity, helping prevent the immune system from attacking healthy skin cells.

Examples of immunosuppressants that may be prescribed include methotrexate, azathioprine, cyclophosphamide, or mycophenolate.

4. Monoclonal antibodies

Monoclonal antibodies may be recommended when other treatments have not successfully controlled symptoms.

Examples include rituximab administered through an intravenous infusion or omalizumab given as a subcutaneous injection.

Care instructions

Self-care measures can help support medical treatment, including:

• Avoiding scratching or rubbing the affected skin;

• Applying cold compresses to help relieve itching;

• Avoiding excessive sun exposure and use sunscreen with SPF 30 or higher on exposed skin;

• Using moisturizing creams recommended by a dermatologist;

• Wearing loose-fitting cotton clothing;

• Washing hands thoroughly with water and mild soap before and after applying corticosteroid cream or ointment.

Blisters should not be intentionally popped, as this can increase the risk of infection and complications.

If drainage is necessary, it should be performed by a dermatologist in a clinical setting using sterile techniques.

Can bullous pemphigoid be cured?

Although bullous pemphigoid can be difficult to treat, remission is possible with appropriate therapy prescribed by a dermatologist.

The duration of the condition varies depending on the person’s overall health and may last for weeks, months, or even years.

Possible complications

The main complications of bullous pemphigoid include:

• Bacterial skin infections;

• Sepsis (a widespread infection in the body);

• Viral infections, including herpes simplex or herpes zoster.

Immediate medical care should be sought if symptoms such as redness, warmth, swelling, pus, fever, foul odor, or worsening skin lesions occur.

In addition, treatments used for bullous pemphigoid may also lead to complications due to medication side effects.