Ocular Cicatricial Pemphigoid //
Ocular cicatricial pemphigoid (OCP) is a rare but serious eye disease that is associated with scarring of the conjunctiva and cornea. It is more frequent as one gets older with the peak incidence being between 60 and 70. It may progress slowly or rapidly, but if left untreated, may result in blindness. OCP is an autoimmune disease; it is not an infection or a form of cancer.
Patients with OCP typically present with inflammation and scarring of the conjunctiva - the mucous membrane lining of the inner part of the eyelids and the outer surface of the eyeball. The redness and irritation that is seen in the early stages of this condition are often thought to represent an allergic or irritative conjunctivitis. The chronic course of OCP and the development of conjunctival scarring are unique features of this condition and often help differentiate OCP from other conditions. Progressive scarred over months or years leads to damage to the eyelid margins with in-turning of the margin (entropion), misdirected eyelashes (trichiasis) which may rub on the cornea, and tear duct obstruction that may lead to or aggravate a pre-existing dry eye. Recurrent corneal abrasions, erosions and ulcerations lead to scarring and vascularization of the cornea with loss of vision.
The clinical course of OCP is highly variable. In many cases it progresses very slowly and often goes into remission for many years. At other times significant redness may be seen with rapid formation of scarring between the lids and conjunctiva (symblepharon). Other conditions such as previous chemical eye injury, trauma, prior lid surgery, or certain infections may mimic OCP. The diagnosis is sometimes difficult to make.
Cicatricial pemphigoid is a systemic disease with gum, mouth or throat sores sometimes developing during the same time frame as the ocular flare-up. A history of these problems helps to make the clinical diagnosis. A conjunctival biopsy is often performed in an attempt to confirm the diagnosis pathologically.
The treatment of OCP has two goals: The first is to arrest the conjunctival inflammation and prevent progressive conjunctival and corneal scarring. This is done be using potent systemic medications such as dapsone, methotrexate, mycophenolate mofetil or cytoxan. Co-management with an internist or rheumatologist is necessary since these medications need to be used for an extended period of time and are associated with serious side effects. The benefits of preserving vision with immunosuppressive therapy must be carefully weighed against the risks of serious systemic side effects. Factors such as a patient’s overall health, the severity of the conjunctival inflammation and scarring, and the anticipated rate of progression need to be considered. The second treatment goal is to stabilize and protect the ocular surface. This includes removal of misdirected eyelashes and the correction of various lid abnormalities. The management of corneal surface problems involves the aggressive use of lubricating drops and ointment, topical antibiotics and daily lid hygiene as well as the use of specialized scleral contact lenses. Surgery of the eyelids, conjunctiva and cornea is often less successful in OCP than in other non-inflammatory disorders. Close and regular follow-up care is needed in all patients.