Bullous disease

Bullous disease: Description, Causes and Risk Factors:PrimaryGenetic.
  • Immunological.
SecondaryLichen planus.
  • Mastocytosis.
  • Infective - herpes simplex, impetigo.
  • Chemical/physical burn.
Bullous disease actually refers to a group of skin disorders rather than one specific disease. These diseases are usually considered to be autoimmune in nature. Bullous diseases usually result in the appearance of blisters or the accumulation of fluid beneath two layers of skin that results in raised bumps, according to the AAD (American Academy of Dermatology).The exact reason why bullous disease occur in some people and not in others are unknown, but doctors believe these conditions are genetic in nature. It is also apparent that the elderly are more susceptible to bullous disease than younger people.Bullous diseases may be acquired or induced or they may be autoimmune in origin. The autoimmune bullous skin disorders are all characterized by the presence of autoantibodies that target distinct adhesion molecules of the epidermis and dermal-epidermal basement membrane zone. The consequences of these antibodies are a loss of the targeted protein's adhesive properties, which leads, in turn, to the appearance of blisters and erosions. Like most autoimmune disorders, autoimmune bullous or vesiculobullous skin disorders are more likely to occur in women. Women of childbearing age have the highest risk for developing autoimmune bullous diseases.Symptoms:General symptoms may include:Bullous diseaseBullae which are large blisters, thin-walled sacs filled with clear fluid have the symptoms - 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. Bullous skin diseases are characterized by the presence of blisters or erosions of the skinand mucous membranes.In some bullous disorders such as pemphigus vulgaris,blistering is the primary disease manifestation. In other disorders, such as Lichen Planusor Systemic Lupus Erythematosus (SLE), blisters occur infrequently.Diagnosis:Doctors rely upon blood tests and skin biopsies to diagnose bullous skin diseases.Several skin diseases may present as vesicles or bullae. Immunofluorescent studies are very helpful in differentiating the various disease entities. Familiarity with the procedure and the various kinds of patterns that are specific and non-specific is essential for the practicing dermatologist. Immunofluorescent patterns are particularly helpful in differentiating pemphigus, bullous pemphigoid, cicatrical pemphigoid, herpes gestationis, dermatitis herpetiformis, linear IgA dermatosis and porphyria.Treatment:Treatment is focused on relief of symptoms and prevention of infection. Tetracycline and Minocycline antibiotics are very useful for mild to moderate disease. They do not work on bacteria, but act directly on the immune system. They can be used in combination with potent topical steroid creams for more rapid relief.Oral steroids (prednisone, prednisolone) are the treatment of choice for severe cases. Regular visits will be needed because the dose must be adjusted frequently, and side effects must be monitored. A fairly high dose is needed initially, and once the blisters have stopped appearing, it is slowly reduced over many months or years. As steroids have some undesirable side effects, dermatologists try to reduce the dose as low as possible. If this is done too quickly, the blisters reappear.Often, immunosuppressive agents (Immuran, CellCept, Methotrexate, cyclophosphamide and Neoral) are used in combination with the oral steroids to allow a lower dose. Severe cases are best treated in the hospital to allow expert dressing of the wounds, and intravenous injections of the most potent treatments.Oral corticosteroids are often used to reduce the inflammation of the skin and suppress the immune system functions that cause bullous skin disease. Immunosuppressant drugs also help limit the effects of the immune system, making them effective treatments for some patients.Note: Risks and benefits of the drugs must be clearly discussed with the physician.NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.


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