Ocular cicatrical pemphigoid (OCP) is an autoimmune blistering disease which affects the conjunctiva, the skin, and multiple mucous membranes (oral mucosa, pharynx, larynx, trachea, esophagus, vagina, urethra, and anus). Nearly 75 to 100% of OCP patients suffer from oral lesions while 25% of patients experience skin involvement like face, neck, and scalp.
OCP occurs in all races with female predominance and a female:male ratio of 1.5:3.1. It is an extremely rare condition in children, since only a limited number of cases have been reported in the literature. The age of onset of OCP ranges between 50 and 60 years; however, the precise age of onset might be at a younger age due to the inability of diagnosing the disease in early stages.
To date, no effective topical agent has been found that prevents OCP activity, however, subconjunctival steroid injections or subconjunctival injections of mitomycin C might help in decelerating the disease progression, whereas systemic therapy demonstrates an outcome. Patients experiencing dry eye symptoms should undergo adjuvant treatment using topical lubricants, whereas the inflammation can be controlled through topical cyclosporine and tacrolimus ointment. Several ocular surgical procedures are available including epilation, punctual occlusion, lid surgery, fornix reconstruction, corneal surgery, and cataract surgery; however these surgeries are performed following a complete control of the inflammation.
The reason for ocular cicatrical pemphigoid (OCP) occurrence is anonymous; however, genetic factors and numerous triggers are capable of increasing the likelihood of the onset of OCP. Persons with HLA-DR2, HLA-DR4 (HLA-DR*0401), and HLA-DQw7 (DQB1*0301) are more susceptible to OCP, meanwhile systemic practolol therapy and topical antiglaucoma drugs (such as phospholine iodide, idoxuridine, epinephrine, pilocarpine, humorsol, and timolol) were found to act as triggers of OCP.