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BULLOUS PEMPHIGOID

 

Introduction

Bullous pemphigoid is a blistering skin disease which usually affects middle aged or elderly persons.  Symptoms:  Bullae (large blisters, thin walled sacs filled with clear fluid, greater than 1 cm. diameter)  multiple  usually located on the arms, legs, or trunk  may also occur in the mouth  may weep, crust over  may appear deep below the surface of the skin  may erode the skin, form ulcers or open sores  itching  rashes  mouth sores  gums, bleeding. Characteristically, crops of tense, fluid-filled blisters develop. They may arise from normal-looking or reddened skin, often in body folds. Usually, the skin is very itchy. Although sometimes pemphigoid is localized to one area such as an ankle, it is usually widespread, and occasionally blisters develop all over.  The blisters of bullous pemphigoid usually appear on the areas of the body that flex or move (flexural areas). About one-third of persons with bullous pemphigoid also develop blisters in the mouth. The disorder may occur in various forms, from no symptoms, to mild redness and irritation, to multiple blisters. The cause is not known, but may be related to immune system disorders. Bullous pemphigoid typically occurs in elderly persons and is rare in young people. It is characterized by a pattern of exacerbations and remissions. It may be without symptoms for 5 or 6 years, then suddenly flare up.

 

How is it diagnosed?

The diagnosis is confirmed by taking a skin biopsy of a typical blister. Skin lesion biopsy shows subepidermal blister (a blister that occurs beneath the outer skin layer and the inner skin layers) and immunoglobulin deposits. Under the microscope, the pathologist can see a split between the main layers of the skin, the epidermis, and the dermis. This is thought to occur because antibodies and white blood cells attack the membrane that holds the skin together. It can be thought of as a type of allergy to one's own skin, i.e. one of the autoimmune disorders.

 

What causes it?

Bullous pemphigoid has been defined as a blistering disease of the elderly which often starts with hive-like and itchy red lesions that later develops large tense blisters. Pemphigoid is an affliction of the elderly, occurring predominantly in persons between 60 and 75 years of age. However, the condition can occur in the under forties and occasionally in children. There is no predilection for any racial or geographical groups and there appears to be no predominant HLA phenotype. The sex incidence is equal overall.            Immune phenomena are believed to be involved in the development of bullous pemphigoid. The finding of autoantibodies to the epidermal basement membrane zone has confirmed that it is indeed a distinctive and recognizable disease. Research data suggest a tentative mechanism for the pathophysiology of the blister formation: Antibodies bind to the basement membrane zone and activate complement. Complement components, especially C3a and C5a, attract leukocytes and act as anaphylotoxins, degranulate mast cells, and release inflammatory mediators. Leukocytes are attached to the basement membrane zone and lysosomal enzymes from these cells result in damage to the basement membrane and thus cause the blister formation. Bullous pemphigoid commonly starts with a nonspecific rash on the limbs. This may precede the blisters anywhere from one week to several months. Sudden generalization of the true eruption of pemphigoid follows and most of the body may be affected within a week. Bullous pemphigoid blisters are tense and dome-shaped obtaining a diameter of up to 7 cm. They may arise in erythematous patches or plaques, or may develop on otherwise normal-appearing skin. The blisters are tough and may remain intact for several days. In some, the fluid is reabsorbed and the epithelium settles back in place like a skin graft. Those which do rupture leave erosions which heal rapidly, leaving mild post-inflammatory changes. Sites of predilection include the inner aspect of the thighs, flexor surfaces of the forearms, the axillae, the groin, and the lower abdomen. Mucous membrane lesions occur less frequently and are usually confined to the mouth. Pruritis is often present and is sometimes severe. Untreated pemphigoid runs a chronic often self-limiting course over a number of years. Patients tend to be otherwise in good health except for coincidental diseases or the ordinary infirmities that may accompany old age.

 

How is it treated?

Treatment is focused on relief of symptoms and prevention of infection. Corticosteroids may help. They are given by mouth or by injection. Topical (applied to a localized area of the skin) corticosteroids may also be used on early, localized lesions. Antibiotics such as tetracycline or erythromycin may also be given. For mild cases, Dr. Jacobs first tries a course of erythromycin, tetracycline, or Dynabac antibiotics. Niacin (a B complex vitamin) may also be used to manage this condition. If the pemphigoid is widespread, hospital admission is usually arranged so the blisters and raw areas can be expertly dressed, and the effect of treatment assessed. Infection also needs to be treated. More severe cases of bullous pemphigoid are treated with steroid tablets, usually prednisone. Corticosteroid therapy has lowered the mortality considerably. Few deaths are attributable to the complications of treatment. The dose is adjusted until the blisters have stopped appearing, which usually takes several weeks. The dose of prednisone is then slowly reduced over many months or years. As steroids have some undesirable side effects, we try to reduce the dose as low as possible. If this is done too quickly, the blisters may reappear. If the pemphigoid does not clear up with steroids alone, several other medicines are useful. These include tetracycline antibiotics, dapsone, azathioprine and methotrexate. Potent steroid creams may help clear up early blisters. Treatment is usually needed for several years, but generally after a few months it is possible to reduce the dose of prednisone to reasonably low levels. In most cases the pemphigoid eventually completely clears up and the treatment can be stopped. Prevention: There is no known way to prevent this disorder.

 

Expectations (prognosis)

Bullous pemphigoid is a relatively benign disorder that tends to be chronic, with flare-ups after long remissions. Infection of the skin lesions is the most common complication. Although bullous pemphigoid can be a chronic condition, it frequently undergoes spontaneous remission, much to the delight of both patient and physician.

 

 

How do I  take care of blisters?

 

Supplies needed?  Ointment: Antibiotic ointment is a topical antibiotic available here.

                                    Band-Aids or hypoallergenic paper tape.

                                    Gauze: Cotton gauze or cotton balls.

                                    Saline: Available at your pharmacy.

 

Brief routine             Twice a day: Pour Saline onto the wound or blister.

                                    Twice a day: Lightly rub the area with a Saline soaked gauze.

                                    Twice a day: After the Saline, liberally apply antibiotic ointment with a Q tip.

                                    Twice a day: If you desire, you may cover with a new bandage.

                                    It's best to keep the wound continuously moist with Antibiotic Ointment.

                                    Severe wounds should not be exposed to excessive water.

                                    ¼ While showering, a thick film of antibiotic ointment will prevent water exposure.

                                    ½ Prevent infection by keeping your fingers off the wound. Please avoid picking.

 

 
Ken Alpern, M.D. Charity Morris, PAC Eleni Litras,
PAC

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Hello, and welcome to our dermatology office. My name is Randy Jacobs, MD, FAAD. Some people are blessed with the most beautiful skin, and it’s all natural. Others have to work at it. Healthy skin is lovely to behold, comfortable to live in, and a pleasure to touch.
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