Bullous Pemphigoid Definition
A disease characterized by tense blisters on the skin. The condition is caused by antibodies that accumulate abnormally in a layer of the skin called the basement membrane. The antigen of this autoimmune disease is localized to the hemidesmosome. Most cases of the disease are in older people but the disease can affect younger people, even babies. Bullous pemphigoid can be chronic, mild and not affect the general health or it can be severe and compromise the health of the sufferer. The diagnosis can be confirmed by a skin biopsy showing the abnormal antibodies deposited in the skin layer. Treatment of mild, limited disease is with topical corticosteroid creams, but especially severe cases may sometimes require high doses of orally administered cortisone-like drugs (corticosteroids) or other immune suppression drugs.
What is bullous pemphigoid?
Bullous pemphigoid is an uncommon skin disease characterized by tense blisters on the surface of the skin. Occasionally, the inner lining tissue of the mouth, nasal passages, or conjunctivae of the eyes (mucous membrane tissue) can be involved. The condition is caused by antibodies and inflammation abnormally accumulating in a particular layer of the skin or mucous membranes. This layer of tissue is called the "basement membrane." These antibodies (immunoglobulins) bind to proteins in the basement membrane called hemidesmosomal BP antigens and this attracts cells of inflammation. The mucous membrane disease is also referred to separately as mucous membrane pemphigoid.
A majority of those affected by bullous pemphigoid are 50 years of age or older. While the cause is unknown, it is felt by some that an aging immune system may become activated in certain individuals with a genetic predisposition to develop bullous pemphigoid.
What are symptoms of bullous pemphigoid?
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, peeling away of affected inner lining tissues, 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. The disease typically worsens and improves over time.
How is bullous pemphigoid diagnosed?
Bullous pemphigoid is often diagnosed by clinical examination and is confirmed by the results of a biopsy of involved tissue. The biopsy with traditional pathology evaluation can demonstrate inflammation of the affected skin levels. Additional testing of the biopsy specimen for antibody immune deposits can reveal the abnormal antibodies located in the basement membrane layer of skin or mucous membrane tissue. Blood testing for circulating basement membrane antibodies can also be helpful.
Bullous pemphigoid-like condition can sometimes be associated with other illnesses, including systemic lupus erythematosus (SLE or lupus) and cancer.
Medically Reviewed by a Doctor on 4/15/2015