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Cicatricial Pemphigoid

(Mucous Membrane Pemphigoid; Ocular Cicatricial Pemphigoid; Benign Mucous Membrane Pemphigoid)

By Melvin I. Roat, MD, FACS

Cicatricial pemphigoid is an autoimmune disorder that scars the conjunctiva and cornea.

  • The eyes become red, then the conjunctiva scars, eyelashes turn inward, and the cornea becomes clouded, blocking vision.

  • The diagnosis is usually based on symptoms and the results of an eye examination.

  • Artificial tears can be used, inwardly turned eyelashes can be removed, and some people require drugs that suppress the immune system.

Cicatricial pemphigoid is an autoimmune disorder (a malfunction of the body's immune system that causes the body to attack its own tissues) that causes inflammation that begins in the conjunctiva (the membrane that lines the eyelid and covers the white of the eye). Both eyes are affected, becoming red at first. Later, the two layers of the conjunctiva may stick together, making it difficult to pull the upper or lower eyelid away from the eye. The eyes become dry. The cornea (the clear layer in front of the iris and pupil) can become opaque, preventing light from reaching the retina and decreasing vision. The conjunctiva can scar and shrink, causing eyelashes to turn inward and further damage the cornea.

Doctors usually base the diagnosis on the person's symptoms and the results of an eye examination, including an examination with a slit lamp (an instrument that enables a doctor to examine the eye under high magnification). People can use artificial tears and doctors can remove inwardly turned lashes (for example, by pulling, freezing, or using electricity) to help relieve symptoms and prevent some complications. But if damage progresses, a drug that suppresses the immune system, such as dapsone or cyclophosphamide, is needed.

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