Bullous Pemphigoid

What is Bullous Pemphigoid?

Bullous pemphigoid is a disease characterized by blisters under the subepidermal space. The subepidermal space is located between the skin and thin layer of fat that covers your muscles. After the blisters develop, they become inflamed and extremely painful. This disorder can last between several months or years. Between outbreaks, the disorder can go into remission and then reappear. If left untreated, the disease can lead to debilitating and even fatal symptoms.

This disorder is most common in people who are over the age of 60, but it can appear in younger people as well. This is considered an autoimmune disorder and is treated much like other autoimmune disorders.

It is common thought that the disorder is caused by a reaction from taking specific medications. However, the full reason it develops is not fully understood.

What are the Symptoms of Bullous Pemphigoid?

There are multiple clinical distinctions for this disorder and is presented in many ways outlined below.

Generalized Form

This is the most common presentation of this disorder. The bullae, or blisters, can appear on any part of the skin’s surface. Typically, the blisters develop in joints before the spread to other parts of the skin.

Vesicular Form

This is a less common presentation of the disorder’s symptoms. The disorder appears as small groups of blisters that are tightly packed together. Typically, these blisters appear in a urticarial or erythematosus base.

Generalized Erythroderma Form

This specific presentation is very rare to see. When the blisters appear, they can closely mimic psoriasis or standard dermatitis, or skin irritation. Typically, this is characterized by blisters or papules that flake and peel.

Urticarial Form

Many patients with Bullous pemphigoid is first present with persistent blisters that eventually turn into bullous blisters. Some patients only show these symptoms and do not develop any others.

Nodular Form

This is an extremely rare form of the disorder. It is usually called pemphigoid nodularis by physicians. The visual signs of this disorder typically shows blisters that come up on normal skin or nodular type skin lesions.

Acral Form

This specific form of the disorder appears in childhood. It is usually associated with standard childhood vaccinations. The blisterous lesions usually appear on the face, soles of the feet, and palms of the hands.

Infant Form

Infants that are affected by this disorder usually have blisters on the soles of the feet, palms of the hands, and on their face. Rarely, these blisters appear in the genital area. Only 60% of the infants affected by this disorder develop generalized blisters over their body.

Vegetative Form

This is a very uncommon form of the disease. It is characterized by vegetating plaques in folds of the skin. Most commonly they are found under the arm, under then neck, and in the folds of the groin.

Bullous Pemphigoid Causes

Bullous Pemphigoid is the result of an autoimmune response where the proteins attaching the top and bottom layers of skin are attacked. Doctors do not fully understand what can trigger this type of response, but some think there could be a genetic predisposition towards it.

While there are many cases of bullous pemphigoid with no specific trigger, there are some cases that can be triggered by medical treatments for other conditions.

The types of medical treatments include:

  • Prescription medications like penicillin, etanercept, sulfasalazine, and furosemide
  • Ultraviolet light therapy used for certain skin conditions
  • Radiation therapy as part of cancer treatment

The triggers of bullous pemphigoid may not be completely understood, but there are some people who are more at risk than others of developing it. Adults over the age of 60 are more likely to develop it over any other age group, and the risk only increases with age. People diagnosed with autoimmune disorders like lupus or with cancer are also more likely to develop this condition.

How is Bullous Pemphigoid Treated?

The main focus of treatment is to allow the blisters to heal as fast as possible. This is because the primary complication of this disorder is the blisters becoming infected, which leads to sepsis. Usually, treatment involves the following:

Corticosteriods: This medical steroid helps the body to heal faster that it typically will on its own.

Immuno-suppressant Drugs: These medications help suppress the immune response, which prevents your body from creating more lesions. Since the reason for the disorder’s development is an immune system over reaction, the body is able to heal faster.

Anti-Inflammatories: Anti-inflammatory drugs can help reduce the swelling of the affected area. This allows for better blood flow and easier delivery of medications to the affected area.

Bullous Pemphigoid Prevention

There is no known way to prevent bullous pemphigoid at this point in time. Once it is diagnosed, the best means a person has of preventing future occurrences is following the medical treatment plan agreed upon by them and their doctor. Even following that plan may not prevent it from happening again, though, as this is a chronic condition and can flare up at any point. Usually, people go into remission after a few years of treatment and remain symptom-free.

People diagnosed with autoimmune disorders should follow the medical treatment plan they have set with their doctors for their disorder and seek immediate treatment if they see symptoms appear. The same is true for those undergoing ultraviolet or radiation therapy.

Last Reviewed:
September 17, 2016
Last Updated:
November 22, 2017