Guide To The Causes And Risk Factors Of Bullous Pemphigoid

Bullous pemphigoid is a type of chronic autoimmune condition that produces large blisters and welts on the skin. While the disorder can affect individuals of any age, it is most frequently diagnosed in patients over sixty years old. The skin changes associated with this condition can occur in small, isolated patches or across larger areas of the body. The armpits, groin, abdomen, and other flexible areas of the body are the most common sites. In addition to skin changes, up to one-third of patients with this condition will experience blisters in the mouth, throat, or esophagus. Symptoms are normally intermittent, and bullous pemphigoid typically resolves after a period of five years. To diagnose bullous pemphigoid, dermatologists perform a visual inspection of the skin, and blood tests and skin biopsies might be necessary. Treatment is usually centered around oral or topical corticosteroids, and these may be supplemented with antibiotics and anti-inflammatory medicine. Patients with severe cases might need to take immunosuppressants too.

The major causes and risk factors for bullous pemphigoid are outlined below.

Immune System Malfunction

Immune system malfunction is believed to be the primary cause of bullous pemphigoid. In a healthy patient, the immune system manufactures antibodies that protect the body from viruses, bacteria, and foreign substances. If an individual develops an immune system malfunction, their immune system starts to make antibodies to attack particular tissues in the body that are not actually a threat. Patients who have bullous pemphigoid normally produce unnecessary antibodies that attack the fibers connecting the outermost layer of skin (the epidermis) to the layer directly underneath it (the dermis). This process triggers an inflammatory response that results in itching, blister formation, and other symptoms. Corticosteroids help reduce the inflammation caused by a malfunctioning immune system, and they can also relieve itching. Immunosuppressants can reduce the activity of the immune system, providing relief to patients who have had a particularly serious immune system malfunction.

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Psoriasis

Like bullous pemphigoid, psoriasis is an autoimmune condition that affects the skin, and it may trigger bullous pemphigoid for certain patients. Psoriasis accelerates the normal lifespan of skin cells, and this results in an abnormal, rapid accumulation of extra cells on the skin's surface. These extra cells form heavy, red scales known as plaques, and patients may also experience joint pain, thick or pitted nails, itching, and dry skin. This condition normally comes and goes in periods known as flare-ups, and some patients may have years in complete remission. To diagnose this disorder, doctors ask the patient about their symptoms and perform a physical exam of the skin, hair, and nails. In most cases, the physical exam provides sufficient information for the doctor to make a diagnosis. A skin biopsy may be used for confirmation.

Treatment options for psoriasis include topical corticosteroids, vitamin D analogs, anthralin, and topical retinoids. These medicines help decrease skin redness and inflammation, and they can also reduce the growth rate of new skin cells. Patients who have severe forms of psoriasis that have not responded to these treatments may need to try methotrexate, cyclosporine, biologics, or other systemic treatments.

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Rheumatoid Arthritis

Rheumatoid arthritis is another autoimmune condition that can trigger bullous pemphigoid. Patients with rheumatoid arthritis typically experience swelling and pain in the joints, and the small joints in the fingers and toes are often affected first. Stiffness may be present in the joints, and this is usually worse in the morning. Some patients may notice a loss of appetite, and the condition can also cause fevers and fatigue. To diagnose this form of arthritis, doctors carry out a physical examination to check the joints for swelling, warmth, and pain. The patient may also have their reflexes and muscle strength assessed. Blood tests to check for elevated levels of erythrocyte sedimentation rate and C-reactive protein are commonly performed, and physicians may request further tests to detect rheumatoid factor and anti-cyclic citrullinated peptide antibodies. MRI scans, ultrasounds, and x-rays can be helpful in evaluating the severity and progression of this condition. Treatment typically consists of corticosteroids, disease-modifying antirheumatic drugs, or biologics. Abatacept and tofacitinib are some of the newest biologic medicines available to treat this form of arthritis, and they are often more effective when taken with methotrexate.

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Certain Medications

Certain medications can increase a patient's risk of developing bullous pemphigoid. Penicillin, etanercept, sulfasalazine, and furosemide are a few of the medicines doctors have currently identified as potential triggers for this condition. Patients who already have other risk factors for bullous pemphigoid, including an underlying condition such as diabetes, rheumatoid arthritis, psoriasis, ulcerative colitis, or multiple sclerosis, should ask their healthcare provider about how they might be impacted by taking these medicines and if a different medication could be prescribed instead. This is particularly important for individuals who are over sixty years old. Potential side effects of penicillin include reduced urine output, bloody diarrhea, weakness, easy bruising, confusion, and agitation. Individuals taking etanercept could experience heartburn, changes in weight, and liver problems such as jaundice or upper abdominal pain. Sulfasalazine use might result in stomach upset, decreased appetite, and dizziness, and some patients using this medicine have developed kidney issues. Furosemide, a diuretic, may lead to constipation, blurred vision, vertigo, and stomach cramps. Rarely, patients using furosemide could experience a reduction in thyroid hormones, and an excessive loss of water and electrolytes may occur. Individuals using any of these medicines should report rashes and skin blisters to their physician as soon as possible.

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Ultraviolet Light Therapy

Ultraviolet light therapy, also known as phototherapy, is commonly used to treat a variety of skin conditions, and it is a recognized trigger for bullous pemphigoid. Patients may be asked to have ultraviolet light therapy as a treatment for eczema or vitiligo, and it is sometimes recommended as a treatment method for jaundice and for mental health conditions such as seasonal affective disorder. During a phototherapy session for a skin condition, the patient may need to apply a special oil to the affected site. After exposing the area to be treated, the patient stands in a cabinet surrounded by ultraviolet lights. The treatment provider activates the lights for a very short period ranging from a few seconds to a few minutes. Patients may need to have treatment sessions several times a week over a course of many months, and the lights will normally be activated for an increasing amount of time at each session. Individuals at risk of bullous pemphigoid should ask their doctor about the risks and benefits of phototherapy for their particular condition. They should also ask about the availability of other options that could provide similar treatment outcomes.