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Bullous Pemphigoid
Bullous pemphigoid is a skin disease characterized by tense blistering
eruptions on the surface of the skin. Occasionally, the inner lining
tissue of the mouth (mucous membrane tissue) can be involved. The
condition is caused by antibodies and inflammation abnormally
accumulating in a certain layer of the skin or mucous membranes.
This layer of tissue is called the ‘basement membrane’ a thin
membrane upon which is posed a single layer of cells. The basement
membrane is made up of proteins held together by type IV collagen.
The epithelial cells are anchored with hemidesmosome to the
basement membrane. The end result resembles a layer of tiles
attached to a thin sheet.
These antibodies (immunoglobulins) bind to proteins in the
basement membrane called hemidesmosomal BP antigens and this
attracts cells of inflammation. The antigen of this autoimmune
disease is localized to the hemidesmosome.
A majority of cases of bullous pemphigoid occur in people 50 years
of age or older. Most cases of the disease are in older people but the
disease can affect younger people, even babies.
While the cause is unknown, it is felt by some that an aging immune
system may become activated in certain individuals with some genetic
predisposition to develop bullous pemphigoid. Bullous pemphigoid
can be chronic, mild and not affect the general health or it can be
severe and compromise the health of the sufferer.
Chronic blistering disease characterized by the eruption of tense,
subepidermal bullae on erythematous or normal skin. Autoantibodies
to the epidermal basement membrane zone are found on direct
microscopy.
The diagnosis can be confirmed by a skin biopsy showing the
abnormal antibodies deposited in the skin layer. Treatment is with
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topical cortisone creams, but especially severe cases may sometimes
require high doses of cortisone-like drugs (steroids) or immune
suppression drugs, such as azathoprine.
Symptoms
Symptoms of bullous pemphigoid include intense itching and
burning sensation of the skin. When the mucous membranes of the
mouth are affected, it can cause pain, burning, and sensitivity to
acidic foods. Eating can be difficult, and involvement in the deeper
areas of the throat can cause coughing. Involvement of the inner
nose can cause nosebleeds.
Diagnoses
Bullous pemphigoid is diagnosed based on the results of a biopsy of
involved tissue. The biopsy can reveal the abnormal antibodies
deposited in the basement membrane layer of skin or mucous
membrane tissue.
Bullous pemphigoid-like condition can sometimes be associated with
other illnesses, including systemic lupus erythematosus (SLE or
lupus) and cancer.
Treatment
Bullous pemphigoid can be chronic and mild without affecting the
general health of affected individuals. Treatment of bullous
pemphigoid can resolve with topical cortisone creams but sometimes
requires high doses of cortisone (‘steroids’) taken internally. Severe
bullous pemphigoid can also require immune-suppression drugs such
as azathioprine . Tetracycline has been used as a treatment option.
Other treatments that have been used for severe disease include
intravenous immunoglobulin infusions, typically given monthly.
Recent research has indicated that large quantities of high-potency
topical corticosteroids applied to the entire body surface were safer
and more effective in controlling extensive bullous pemphigoid than
oral corticosteroids. It was felt by the researchers that topical
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corticosteroids should now be the treatment of choice for bullous
pemphigoid, particularly when the disease is not extensive.
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