Bullous diseases of the skin, include bullous pemphigoid, pemphigus vulgaris, pemphigus vegetans, pemphigus foliaceous, paraneoplastic pemphigus, mucous membrane pemphigoid, linear IgA bullous disease, dermatitis herpetiformis, and epidermolysis bullosa acquisita. The hallmark of these diseases is the development of blisters. Blisters are the accumulation offluid between cells in the upper layers of the skin, specifically the epidermis (top layers) or dermis (layer beneath the epidermis). The type of disease depends upon what level in the skin the blisters form and where they are located on the body. Many of these diseases are also categorized as autoimmune diseases in which the body’s immune system, the system that protects and defends the body, malfunctions (breaks down) and attacks the body’s own tissues.
The Cause of Autoimmune Diseases The cause of autoimmune diseases is unknown, but genetic make-up and environmental exposures may be factors. None of these diseases are contagious, however, certain medications may trigger the development of these disorders. They are more common in the elderly.
Bullous pemphigoid is made up of blisters that do not break easily. They develop on the abdomen, groin, inner thighs, arms, and occasionally on the mouth, eyes, and nose. The blisters form under the epidermis at the junction where the epidermis and dermis meet. Bullous pemphigoid may begin with itching, a rash, or hives, typically over the entire body or in one area. The disease may last for several months to years.
Pemphigus vulgaris is the most common type of pemphigus. Flaccid (soft and limp) blisters appear on healthy-looking skin and mucous membranes, especially in areas of pressure. These blisters form within the epidermis, high in the skin, and may be painful. Skin may be “peeled” off by rubbing a finger on it (Nikolsky sign). The blisters heal with darker spots (postinflammatory hyperpigmentation). Pemphigus occurs frequently during middle age, and may last for years.
Pemphigus vegetans is a variant of pemph igus with thick erosions (sores) in the groin and under the arms.
Pemphigus foliaceous, another variant of pemphigus, is more itchy than other autoimmune blistering diseases. The face and chest are the most common sites affected. It can involve the skin anywhere on the body, but the blisters rupture easily leaving shallow erosions. There may also be loose, moist scales on the skin.
Paraneoplastic pemphigus is the most rare and serious type of pemphigus because it is associated with an underlying tumor. Special tests may be needed to identify this type of autoimmune blistering disease.
Mucous membrane pemphigoid has blisters and erosions thai affect mucous membranes like the mouth, nose, eyes, throat, and genitals. It may last for years and usually heals with scarring. It may also be associated with burning and itching.
Linear bullous disease has blisters that are very itchy and often occur in a ring-shape. It is unassociated with internal problems and IS prevalent in middle age.
Dermatitis herpetiformis affects the elbows, knees, neck, and buttocks with symmetrical (same on both sides) tiny blisters, bumps, and erosions. There is severe itching, and the condition is associated with gluten-sensitive bowel disease. Deposits of the immunoglobulin IgA in the skin help make the diagnosis. It is primarily seen in younger people.
Epidermolysis bullosa acquista (ERA) IS an autoimmune reaction to the patient’s own collagen anchoring fibrils. There is also anti- bodies in the blood. EBA has symptoms that include itching, blisters, and/or hives. The hands and feet are more commonly affected. The lesions heal with scarring and tiny milia (cysts). It is prevalent in middle age.
· A complete history and physical exam noting the appearance and location of the lesions.
· Skin biopsy, a procedure in which a small piece of skin is taken to be examined under a rnicroscope.
· Direct immunoflourescence, a special skin biopsy, that examines tissues by staining it, and patterns of deposits of different immune substances in the body can be seen. These patterns correlate with the different bullous diseases.
· Indirect immunofluorescence, a special blood test, that measures antibodies (substances in the blood directed against other substances).
· Oral corticosteroids (prednisone) are antiinflammatory medicines which suppress the immune system. Large doses over long periods of time may be needed; however, lowering the dosage, when possible, will decrease side effects.
· Immunosuppressant drugs like mycophenolate mofetil, cyclophosphamide, or azathioprine may be added to allow the dosage of prednisone to be decreased and enhance treatment.
· Tetracycline may be beneficial in bullous pemphigoid.
· Sulfa drugs may be used to treat dermatitis herpetiform is and linear IgA bullous disease.
Bullous diseases are controllable, therefore, it is important to see a dermatologist at the first sign of these diseases in order to make a proper diagnosis and get early treatment.