Bullous Disease
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Bullous diseases of the skin include bullous pemphigoid, pemphigus vulgaris, pemphigus vegetans, pemphigus foliaceous, paraneoplastic pemphigus, mucous membrane pemphigoid, linear IgA bullous disease, dermatitis herpeti-formis, and epidermolysis bullosa acquisita.The hallmark of these diseases is the development of blisters. Blisters are the accumulation of fluid between cells in the upper layers of the skin, specifically the epidermis (top layers) or dermis (layer between 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 Disease
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 drug eruption
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 (top layers of the skin) at the junction where the epidermis and dermis (lower layers of the skin) 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.
Bullous pemphigoid on trunk and extremities
Bullous pemphigoid on the hand
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 (post-inflammatory hyperpigmentation). Pemphigus occurs frequently during middle age, and may last for years.
Pemphigus vulgaris on the back
Pemphigus vegetans is a variant of pemphigus with thick erosions (sores) in the groin and under the arms.
Pemphigus vegetans on groin
Pemphigus foliaceous, another variant of pemphigus, is more itchy than other auto-immune 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.
Pemphigus foliaceous
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.
Paraneoplastic pemphigus
Mucous membrane pemphigoid has blisters and erosions that affect mucous membranes like the mouth, nose, eyes, throat, and genitals.
Mucous membrane pemphigoid
It may last for years and usually heals with scarring. It may also be associated with burning and itching.
Linear IgA 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.
Linear IgA bullous disease
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.
Dermatitis herpetiformis
Epidermolysis bullosa acquista (EBA) is an autoimmune reaction to the patient's own collagen anchoring fibuls. 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.
Diagnosis Includes:
- 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 microscope.
- 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).
Treatment
- Oral corticosteroids (prednisone) are anti-inflammatory 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 myco-phenolate 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 herpetiformis 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.
To learn more about bullous disease, call toll-free (888) 462-DERM (3376) to find a dermatologist in your area.
AAD Web site: www.aad.org
Toll-free: (888) 462-DERM (3376)
Images used with permission of the American Academy of Dermatology
National Library of Dermatologic Teaching Slides
©2005 American Academy of Dermatology
Revised 2004
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