Bullous Pemphigoid
William C. Shiel Jr., MD, FACP, FACR
Dr. Shiel received a Bachelor of Science degree with honors from the University of Notre Dame. There he was involved in research in radiation biology and received the Huisking Scholarship. After graduating from St. Louis University School of Medicine, he completed his Internal Medicine residency and Rheumatology fellowship at the University of California, Irvine. He is board-certified in Internal Medicine and Rheumatology.
Melissa Conrad Stöppler, MD
Melissa Conrad Stöppler, MD, is a U.S. board-certified Anatomic Pathologist with subspecialty training in the fields of Experimental and Molecular Pathology. Dr. Stöppler's educational background includes a BA with Highest Distinction from the University of Virginia and an MD from the University of North Carolina. She completed residency training in Anatomic Pathology at Georgetown University followed by subspecialty fellowship training in molecular diagnostics and experimental pathology.
What is bullous pemphigoid?
Bullous pemphigoid is an uncommon skin disease characterized by tense blistering eruptions 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 (exacerbates) and improves (remits) over time.
How is bullous pemphigoid diagnosed?
Bullous pemphigoid is diagnosed based on 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.
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