Ocular Cicatricial Pemphigoid

Posted by Elaine Moore Apr 10, 2007

Ocular cicatricial pemphigoid (OCP) is a systemic autoimmune pemphigoid disorder that has both ocular and non-ocular manifestations. Ocular cicatricial pemphigoid can cause bullous lesions of the skin and mucous membranes that result in scarring of the affected skin, conjunctiva (inner lining of the eye), and other mucous membranes.

In some cases, conjunctival involvement may emerge up to 10 years before other mucosal or skin lesions develop. Alternatively, ocular cicatricial pemphigoid may exclusively affect the conjunctiva with no other lesions occurring. In cases where bullous lesions and scarring over the esophagus or trachea constrict these organs, ocular cicatricial pemphigoid disorder can be fatal.

Who is Affected?

Ocular cicatricial pemphigoid occurs in 1 in every 15,000-20,000 persons. Females are affected twice as often as males and all races are affected. Ocular cicatricial pemphigoid primarily affects people in their 60s although it can occur as early as age 30. Because the early symptoms are often difficult to diagnose, most people are probably not diagnosed until the disease is advanced.

OCP is believed to occur as a result of a type 2 hypersensitivity reaction in people who are genetically predisposed. Susceptibility is linked to the immune system gene HLA-DQw7. Environmental triggers suspected of initiating the hypersensitivity response include chemicals and infectious agents. A drug-induced form of OCP has been linked to eye drops containing practolol pilocarpine timolol echothiophate iodide epinephrine.

Symptoms

Eye changes in OCP include an early unilateral (affecting one eye) chronic form of conjunctivitis that eventually affects both eyes. Stage 1 includes mild conjunctivitis and fibrosis characterized as a fine white striae or fine line. Stages 2-4 include structural changes that progress to dry eye with keratinization of the cornea.

Non-ocular symptoms include blisters affecting the skin, scalp, mouth, nose, pharynx, larynx, trachea, esophagus, vagina, urethra, and anus.

Immune System Changes

In ocular cicatricial pemphigoid, chronic inflammation causes the basal epithelium to separate from the basement membrane. This results in an underlying layer of epithelial cells that forms scar tissue. Immunoglobulin antibodies and complement are found in the epithelial basement membrane zone of the conjunctiva. This finding is used to diagnose OCP. Circulating antibodies to conjunctival epithelium and anti-nuclear antibodies (ANA) are usually found in patients with OCP. Patients with OCP also show elevated levels of soluble CD 8 glycoprotein from increased circulating T helper cells and elevated levels of tumor necrosis factor (TNF).

Treatment

Treatment for OCP depends on the areas that are affected and the severity of the lesions. Current therapies include corticosteroids, Dapsone, Methotrexate, Azathioprine, and Cyclophosphamide.

Resources:

N Tesavibul, Multiple Autoimmune Diseases, The Ocular Immunology and Uveitis Foundation, accessed March 20, 2007.