Dr. M.J. Bazos, MD. Patient Handout

PEMPHIGOID, BULLOUS

About Your Diagnosis
Bullous pemphigoid is a chronic bullous disease of the skin and mucous membranes. It typically begins as an eruption of red wheals (urticarial lesions) and evolves over weeks to months to bullae (large blisters). Bullous pemphigoid is an autoimmune disorder (i.e., a condition in which your immune system mistakenly attacks normal parts of the body, resulting in tissue injury or disease). It equally affects men and women and typically affects adults older than 60 years. It may occur in children. This condition may be hereditary but is not infectious or cancerous. Diagnosis is usually based upon the appearance of skin lesions, evaluation of skin specimens, and blood tests. Your doctor may perform a skin biopsy (i.e., removal of a small piece of skin or other tissue) for laboratory evaluation to assist in diagnosis. Medications that suppress your immune system have significantly improved symptoms and lessened the severity of the disease. However, bullous pemphigoid can persist for a long time, or it can recur.

Living With Your Diagnosis
Skin lesions typically begin as an eruption of red wheals (urticarial lesions) and evolve over weeks to months to bullae (large blisters). The bullae are typically tense and oval or round. They may arise from normal skin or from the red wheals. The bullae may rupture and cause erosions that may be painful. You may experience itching with the skin lesions. Bullous pemphigoid also can develop into erosions of the mouth, throat, anus, and vagina, which may also be mildly to moderately painful. The lesions can be generalized or localized to certain areas of the body. They typically involve the lower legs (often the first place that lesions develop), armpits, inner thighs, abdomen, and forearms. You may have itching and pain, although these symptoms may be absent. Some bullae rupture, resulting in erosions of the skin and mucous linings. These erosions are prone to secondary bacterial infection, especially if you scratch the lesions. These infections often require antibiotic therapy. Significant pain and fever typically do not occur except in severe cases. You may also experience social embarrassment because of your skin’s appearance.


Treatment
Specific treatment depends upon the location and severity of your bullous pemphigoid, its impact on the quality of your life, and your response to therapy. Treatment aims to lessen the severity of your condition and to prevent complications. Treatment consists of general measures and medications.
General measures are as follows:
1. Maintain good skin hygiene to reduce outbreaks and to decrease the risk of secondary bacterial infection.
2. Avoid skin injury, including scratching, which can aggravate bullous pemphigoid and contribute to secondary infections.
3. Individuals with bullous pemphigoid can become depressed or experience other psychological conditions. If you feel depressed or are having difficulty coping with your skin disorder, talk to your doctor about the best treatment options, including psychological counseling.
Your doctor may prescribe a variety of medications to reduce inflammation and symptoms and to lessen the severity and duration of bullous pemphigoid.
These medications include:

1. Topical steroid creams, lotions, and ointments are effective in mild cases of bullous pemphigoid and as combination therapy for more severe cases. Your doctor may recommend placing occlusive dressings over the topical medications to increase their effects. Side effects of topical steroids include skin atrophy, formation of abnormal, small blood vessels, and absorption of medication through the skin into the bloodstream, which can cause toxic effects. To decrease the risk of side effects, do not exceed the recommended dosage prescribed by your doctor.
2. Corticosteroid tablets or injections are effective in treating bullous pemphigoid. Side effects are more likely with higher doses and include increased risk of infection, swelling, ulcers, diabetes mellitus, and osteoporosis (thinning of your bones). Do not stop steroid medications without first consulting your doctor because abrupt cessation of these medicines can result in severe weakness, fatigue, and low blood pressure.
3. Immunosuppressive medications such as azathioprine and cyclophosphamide are potent suppressors of your immune system and are effectivein treating bullous pemphigoid. When these agents are combined with steroids, they allow less steroids to be used, thereby decreasing the risk of side effects. Side effects of these agents include increased susceptibility to infection, and toxicity to your body’s organs including the bone marrow (anemia, low values of white blood cells and platelets), liver, and kidneys. Your doctor will need to closely monitor your response to therapy and to perform laboratory tests to check for possible toxic effects.
4. Your doctor may prescribe dapsone to reduce skin eruptions and blistering and to lessen the severity of your condition. You will probably need to take this medication for an extended period. Your doctor will need to monitor for side effects by checking periodic laboratory tests. Side effects may include breakdown of red blood cells (hemolytic anemia), inflammation of the peripheral nerves (peripheral neuropathy), nausea, vomiting, and abdominal pains.

No specific dietary measures can prevent or treat bullous pemphigoid. In severe cases, lesions in the lining of your mouth or throat cause pain with eating or swallowing. You may need to follow a liquid or soft diet to ensure adequate nutrition. You will need to aggressively cleanse and monitor your wounds to prevent complications such as infection. Your doctor may recommend one or more of the following measures to care for your bullous pemphigoid:
1. Cleansing baths.
2. Local cleansing of skin wounds.
3. Wound dressings including topical steroids and use of antibiotic ointments.

The DOs
• Take medications as prescribed by your doctor.
• Inform your doctor of all other medications, including over-the-counter medicines, that you are taking. Continue these medications unless your doctor instructs you to stop them.
• Read the labels of medicines and follow all instructions. Consult your doctor if you have any concerns, or if you have new or unexplained symptoms that may result from side effects of the medication.
• Eat a well-balanced, nutritious diet. If lesions in your mouth or throat are causing pain with eating or swallowing, follow a liquid or soft diet to ensure adequate nutrition.
• Avoid activities that cause overheating and excessive sweating or moisture. If you perform activities that result in excessive moisture, immediately shower and cleanse the skin lesions.
• Maintain good skin hygiene to reduce the risk of secondary bacterial infection.
• Keep scheduled follow-up appointments with your doctor. They are essential to monitor your condition, your response to therapy, and to screen for possible side effects of treatment.
• Monitor your skin for healing and for evidence of secondary bacterial infection. Signs and symptoms of infection include redness around the skin lesions, purulent discharge (pus), increased pain or swelling of the skin lesions or lymph nodes, and fever.
• Frequently wash clothing, towels, and linens when skin lesions are oozing, crusting, or infected. This action reduces the risk of transmission of infection.

The DON’Ts
• Do not stop your medicine or change the prescribed dose without consulting your doctor.
• Do not exceed recommended doses of medicines, because higher doses may increase your risk of toxic effects.
• Do not use potent topical steroids on the skin of the face or genitals because these areas are most prone to skin injury and atrophy (thinning and wasting of the skin associated with wrinkling, and abnormal, small blood vessels).
• Do not abruptly stop steroids or immunosuppressive therapy, because you may experience a rebound worsening of your condition. Suddenly stopping steroid medication may result in serious health consequences, including severe weakness, fatigue, and low blood pressure. Consult your doctor before stopping these medications.
• Do not drive or perform other potentially hazardous activities when taking medications that can cause drowsiness or sedation (antihistamines or pain medications).
• Avoid activities that can increase the risk of infection of skin lesions.
When To Call Your Doctor
• If you have any signs or symptoms of infection (see above).
• If you notice that lesions are becoming worse, or if new lesions appear despite appropriate therapy.
• If you have signs and symptoms of systemic illness, including fever, lethargy, confusion, or weakness.
• If you have new or unexplained symptoms that may indicate a complication of your condition or side effects from medications.