Bullous Pemphigoid: Information on Causes, Diagnosis & Treatments

Bullous pemphigoid is a condition caused by a malfunctioning immune system that produces large blisters and skin rashes on the abdomen, arms, legs, and in some cases, the mucous membranes. Some blisters appear in the folds of skin like the inner side of the knees, elbows, and shoulders. Usually, the blisters will not rupture upon contact.

While most blisters are filled with clear fluid, some contain blood. The skin surrounding the blisters typically appears darker and more reddened than in other areas. The condition can also cause a hive-like rash or eczema.

Many individuals who suffer from bullous pemphigoid have very itchy skin or develop sores or blisters in the mouth. Usually, the highest concentration of blisters occurs in the mucous membranes of the mouth and eyes. Nearly all blisters will leave some form of scarring.

• Who Is at Risk for Bullous Pemphigoid?
• Common Symptoms
• Clinical Presentations of Bullous Pemphigoid
• Diagnosing Bullous Pemphigoid
• Treating the Condition
• Monitoring the Condition

Who Is at Risk for Bullous Pemphigoid?

While any individual of any age can develop bullous pemphigoid, the condition tends to occur most commonly in individual 60 years and older with the risk increasing as the patient ages. Medical science has yet to determine the exact cause of the condition but believes it is associated with a malfunctioning immune system that typically produces antibodies to ward off viruses, bacteria, and other harmful foreign substances.

Generally, the body's immune system fails to provide protection against bullous pemphigoid by producing antibiotics to ward off the condition between the dermis and epidermis layers of skin. It is believed that the antibodies instead trigger the inflammation that causes bullous pemphigoid blisters. The only noncontributing factors associated with the condition in a certain number of cases involve:

• Exposure to Radiation and Light – Exposure to ultraviolet light therapy for treating skin conditions and radiation for fighting cancers are thought to trigger bullous pemphigoid.


• Taking Specific Drugs – Certain prescription medications are thought to be the likely cause of a bullous pemphigoid condition. These drugs could include Lasix (furosemide), Azulfidine (sulfasalazine), Enbrel (etanercept), and penicillin.


• Allergic Responses


• Lifestyle and Diet Choices


• Infections

Complications

Infection can occur from ruptured blisters that eventually lead to sepsis, a serious blood infection that affects the entire body. Without immediate treatment, sepsis can be life-threatening, especially for the elderly or those who have a compromised health condition. In addition, the patient can also develop streptococcal or staphylococcal skin infections that might be directly associated with the condition or other related diseases.

Bullous pemphigoid sometimes causes scarring in the mucous membranes of the eye or mouth. There also other variable features associated with the condition that includes:

• A developing non-specific rash the tends to last for several weeks prior to the appearance of a blister


• A nummular dermatitis-resembling eczematous area of skin


• Small blisters


• Ring-shaped (annular) lesions


• Urticaria-appearing reddened skin


• Altered pigmentation that appears after affected skin area inflammation has decreased


• Small cysts that appear in healed areas

Common Symptoms

The most common symptoms associated with bullous pemphigoid involve the development of large blisters that contain clear fluid and, at times, blood. These blisters can occur on the lower abdomen, upper thighs, arms, and groin area. However, the blisters mostly tend to develop in the folds and creases of the skin.

Many individuals suffer itchy skin associated with developing sores and blisters. Other associated bullous pemphigoid symptoms include:

• A red rash that develops before the appearance of blisters


• The development of thick blisters that are not easy to rupture


• Large blisters filled with blood or clear fluid


• Painful and sensitive blisters when ruptured


• Eating difficulties when the blisters have developed inside the mouth and throat, which leads to severe coughing


• Nosebleeds associated with the development of blisters in the inner nose the tends to worsen over time

Clinical Presentations of Bullous Pemphigoid

The condition often reveals itself and very distinct presentations that are categorized as:

• Generalized Bullous Pemphigoid


• Vegetative Pemphigoid


• Vesicular Pemphigoid


• Generalized Erythroderma Pemphigoid


• Urticarial Lesions


• Nodular (pemphigoid nodular)


• Acral (child-onset bullous pemphigoid) caused by vaccinations


• Infant Bullous Pemphigoid

Diagnosing Bullous Pemphigoid

Doctors will usually recommend one or more tests to accurately diagnose bullous pemphigoid that might include:

• Histopathological analysis that examines the sub- epidermal blister that usually involves polymorphic inflammatory infiltrates on basophils and mast cells.


• DIF (direct immunofluorescence) studies to identify abnormalities in perilesional skin.


• IDIF (indirect immunofluorescence) studies that test the patient's serum to identify autoantibodies associated with the condition.

Typically, the doctor will confirm a diagnosis by obtaining a small biopsy (tissue sample) of the affected skin that can be analyzed in a laboratory setting under a microscope. The pathologist can validate a diagnosis by viewing the separation of the top layers of skin.

Immunofluorescence staining is often used to quickly identify antibiotics that can be found in the bloodstream. In addition to the above testing, the doctor might also recommend a monitoring and planning treatment to ensure that the patient has the best potential chance of for a recovery.

Treating the Condition

Patient who suffer widespread bullous pemphigoid usually receive care for their blisters in a hospital environment where the doctor will likely recommend a variety of treatments that could include:

• Intensive topical steroids for treating the disease


• Systemic steroids that assist the entire body in the healing process


• Common emollients and topical steroids that relieved dryness and itching


• Steroids-sparing drugs


• Pain relievers


• Antibiotic medications to treat secondary bacterial infections


• Prednisone or another steroid after leaving the hospital

Prednisone medication dosages are usually adjusted to ensure that the patient receives the most successful result in their treatment. Usually, the dose adjustments occur over several weeks until all blistering has stopped. However, the reduction of prednisone often takes many months or years to avoid undesirable side effects. The doctor might also recommend that the patient take other drugs including:

• Tetracycline antibiotics


• Methotrexate


• Dapsone


• Nicotinamide


• Mycophenolate


• Rituximab


• Azathioprine


• Intravenous immunoglobulin

Those who have been taken systemic steroids for even a short duration might also be prescribed additional drugs that can alleviate the symptoms associated with some serious side effects including osteoporosis, diabetes, hypertension, and gastritis.

Monitoring the Condition

Patients who undergo systemic treatment for extended periods usually require numerous follow-up visits with their doctor to ensure the extent and severity of the bullous pemphigoid condition are well-managed. The doctor will consider a variety of factors in assessing and monitoring the patient's condition that includes:

• Affected areas involving mucous membranes and skin


• The severity of itching


• The numbers and kinds of lesions including eczematous plaques, urticarial wheals (plaques), and blisters


• The type of lesion including non-transient and transient


• option patient of the results of active treatments, the need to reduce treatment, minimize treatment, maintain the condition or determine the treatment has resulted in a complete remission.

To properly monitor the condition, the doctor will also evaluate the patient's body weight and blood pressure while lowering blood tests and a DEXA bone scan before making adjustments to medications when necessary.

The individual usually requires treatment for many years. However, some individuals stop taking the medications after experiencing a complete remission of their bullous pemphigoid condition. However, the condition can recur and require further treatment.

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Sources:

http://www.mayoclinic.org/diseases-conditions/bullous-pemphigoid/symptoms-causes/dxc-20157320

http://www.mayoclinic.org/diseases-conditions/bullous-pemphigoid/home/ovc-20157298

https://my.clevelandclinic.org/health/articles/bullous-pemphigoid

https://emergency.cdc.gov/agent/vesicants/tsd.asp