Ocular Cicatricial Pemphigoid
C. Stephen Foster, M.D.
Ocular cicatricial pemphigoid is an auto immune disease affecting the conjunctiva. You will remember, from the first Patient Information entry on this Web Site, that auto immune diseases result from dysregulation of the patient's immune system, with the white blood cells becoming "confused" and beginning to attack not only germs but also part of the patient's own body. In rheumatoid arthritis, for example, the white blood cells become confused and begin to attack tissue in the patient's joints. In pemphigoid, the white blood cells attack skin and mucous membrane, particularly mucous membranes of the mouth, eyes, nose, throat, vagina, and rectum. Patients may or may not have more than one site affected by the disease, but 70% of the patients with cicatricial pemphigoid have eye involvement. And because this is a systemic autoimmune disease, it cannot be successfully treated, long-term, with simple topical (drops) therapy. It must be treated systemically, and typically with strategies that "cool down" the immune system, i.e., suppress the immune system sufficiently to stop the autoimmune process. Suppressing the immune system sufficiently to stop the autoimmune process for a short period (one to five years) is usually sufficient to enduce permanent remission of the disease. If this is not done, then continued inflammation, continued scarring, and eventual blindness in both eyes usually occurs.
The immunsuppressive/immunomodulatory medication which has been found effective by dermatologists and by us in caring for patients with progressive cicatricial pemphigoid is not cancer chemotherapy-type medication that is associated with feeling sick all the time, losing hair, and running the risk of a lethal complication of the treatment itself. Used properly, by an individual who is, by virtue of both training and experience, an expert in the use of such medications, the medications are not only highly effective, but typically quite safe. We have published on this latter point and have shown that, in fact, the use of such medications in our hands is infinitely safer than is the long-term use of systemic cortisone-type medication.
The future holds great promise for patients with a variety of autoimmune diseases including cicatricial pemphigoid. We have identified the "susceptibility gene" for developing this disease, and we have identified the target antigen that gets attacked by the patient's white blood cells when a patient develops cicatricial pemphigoid. We are cloning the gene for that antigen, and hope to develop a diagnostic test which will enable diagnosis of the disease much earlier in its course. Additionally, we are highly optimistic that treatment strategies that re-educate the patient's white blood cells, enabling them to do their job of attacking and killing germs without attacking and damaging the patient's own body tissues is on the horizon.