Cicatricial Pemphigoid

Cicatricial Pemphigoid

Cicatricial Pemphigoid - an autoimmune disorder in which painful blisters form on the inner surfaces of the eyelids (and may form on other mucus membranes, such as in the MOUTH and NOSE). SCAR tissue that forms after the blisters heal continues to irritate the inner eyelids as well as the outer surface of the EYE (sclera and CORNEA). The blisters commonly involve the lacrimal (tear) glands and ducts, reducing tear production and causing DRY EYE SYNDROME. Cicatricial pemphigoid occurs when the body’s IMMUNE SYSTEM produces antibodies that attack the cells that form the mucus membranes. Trauma appears to activate the eruptions of blisters and may be as inconsequential as rubbing the eye or the irritation such as occurs with exposure to environmental particulates such as pollen and dust. Some people first experience outbreaks of cicatricial pemphigoid following eye operations such as CATARACT EXTRACTION AND LENS REPLACEMENT or BLEPHAROPLASTY.

The diagnostic path includes laboratory tests to assess the levels of antibodies in the blood, particularly IMMUNOGLOBULIN G (IGG) and IMMUNOGLOBIN A (IGA), the antibodies most closely associated with cicatricial pemphigoid. Treatment focuses on reducing BLISTER formation and minimizing scarring, typically by taking oral CORTICOSTEROID MEDICATIONS or IMMUNOSUPPRESSIVE MEDICATIONS. As with other AUTOIMMUNE DISORDERS, cicatricial pemphigoid tends to be chronic and recurrent. The persistent irritation can result in damage to the cornea that causes VISUAL IMPAIRMENT and, when severe, results in blindness.

See also ANTIBODY; CONJUNCTIVITIS; CORNEAL TRANSPLANTATION; ECTROPION; HUMAN LEUKOCYTE ANTIGEN (HLA).

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