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Mucous Membrane Pemphigoid

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

By Melvin I. Roat, MD, FACS, Jefferson Medical College, Thomas Jefferson University

Mucous membrane 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 suspected based on symptoms and the results of an eye examination and is usually confirmed by a biopsy of the conjunctiva.

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

Mucous membrane 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).

An Inside Look at the Eye

Symptoms

Both eyes are affected, becoming red at first. Later, the conjunctiva shrinks, making it difficult to pull the upper or lower eyelid away from the eye. Much later, the eyes become dry.

The cornea (the clear layer in front of the iris and pupil) can become cloudy, preventing light from reaching the retina and decreasing vision.

The conjunctiva can scar and shrink, causing eyelashes to turn inward (see Trichiasis) and further damage the cornea.

Diagnosis

  • A doctor's evaluation of symptoms

  • An eye examination

  • A biopsy of the conjunctiva

Doctors usually suspect the diagnosis of mucous membrane pemphigoid based 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). The diagnosis is usually confirmed by a conjunctival biopsy. In this type of biopsy, a sample of the conjunctiva is removed and examined under a microscope.

Treatment

  • Artificial tears

  • Removal of lashes

  • Sometimes drugs

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. If the disease progresses, a drug that suppresses the immune system, such as dapsone, methotrexate, mycophenolate mofetil, or cyclophosphamide, is needed.

Resources In This Article

Drugs Mentioned In This Article

  • Generic Name
    Select Brand Names
  • CYTOXAN (LYOPHILIZED)
  • ACZONE
  • OTREXUP

* This is the Consumer Version. *