What is a Cicatricial Pemphigoid?

Cicatricial pemphigoid is a rare condition characterized by the formation of painful, oozing blisters on the skin, mouth, nose, or eyes. It is considered an autoimmune disorder, meaning that the body's own immune system mistakenly triggers inflammation and blistering on otherwise healthy tissue. Doctors do not fully understand why or how cicatricial pemphigoid begins, but the vast majority of known cases occur in patients over the age of 60. Treatment measures usually involve topical or oral anti-inflammatory drugs and regular wound care to prevent scarring.

Most medical researchers believe cicatricial pemphigoid is a genetic condition. Due to its rarity, few comprehensive studies have been performed to determine its exact causes and triggers. It is clear, however, that problems occur when autoantibodies produced by the immune system start attacking proteins in the body's mucous membranes. Any mucosal lining can be affected, but blisters usually form in the mouth, nostrils, or eyes. It is also possible to experience skin lesions on the face, neck, or genitals.

Blisters typically begin as small discolored spots that may or may not be sore. Over time, blisters on the skin, mouth, or nostrils become open sores that ooze pus and crust over. Painful open lesions can easily become infected, especially when they are located inside the mouth. Cicatricial pemphigoid of the eyes may cause redness, dryness, itching, burning, and light sensitivity. Vision is often affected to some degree, and some people eventually lose sight in one or both eyes.

When a primary care doctor suspects cicatricial pemphigoid, he or she can refer the patient to a dermatologist or an ophthalmologist for a thorough screening. A specialist can collect blood and tissue samples to check for the presence of specific autoantibodies that suggest cicatricial pemphigoid. If the eyes are involved, a slit lamp test may reveal damage to the conjunctiva or retina. After ruling out other more common skin and eye disorders, the doctor can determine the best way to treat the condition.

Cicatricial pemphigoid cannot be cured, but doctors can usually provide symptom relief with medical or surgical care. Skin, mouth, and nose lesions are usually treated with a prescription topical ointment to numb pain and dry out blisters. Oral corticosteroids may be administered to ease inflammation and swelling. Large, painful blisters may need to be aspirated and dressed with medicated bandages.

Patients with eye problems may respond to oral medications and eye drops, but surgery is necessary in many cases to prevent vision loss. If blisters are present on the eyelids, a specialist can aspirate or excise them to prevent eye irritation. When the eye itself is involved, a lens replacement procedure is often needed to remove the damaged lens, clean out blood and damaged tissue, and insert an artificial lens. Ongoing medical care is important with all types of cicatricial pemphigoid to reduce the chances of recurring symptoms.