Ocular cicatricial pemphigoid


A rare chronic autoimmune eye condition that can result in blindness if not treated. The conjunctival and mucosal layers of the eye (inside of eyelid and outside of eye) become progressively inflamed and scarred. The condition can be very difficult to treat.


* Conjunctival inflammation * Conjunctival scarring * Peripheral corneal thinning * Dry eye * Impaired vision


Patients may or may not have more than one site affected by the disease. However, 70% of the patients with CP have eye involvement. At the onset (stage I), only one eye is typically affected. Symptoms of conjunctivitis develop: irritation, burning, tearing, mucoid discharge and red eye (Figure 1). These symptoms usually continue, but may stop. Eventually, the condition worsens with development (usually within 3 to 4 years) of a similar conjunctivitis in the other eye. The disease progresses to conjunctival scarring and shrinkage (stage II), (Figure 2). As the disease worsens, adhesions called symblepheron form (Stage III) (Figure 3). In the end (Stage IV), untreated CP progresses to profound dry eyes, inability to move the eye due to immobilization from ankyloblepheron (adhesions forming between the upper and the lower eyelids) and blindness of both the eyes due to corneal scarring (Figure 4). It may take the untreated disease 10 to 20 years or more to reach the end stage, with bilateral blindness the result. The more advanced the disease, the more likely it is to progress significantly within the next 2 years.


A thorough history and eye and systemic examination are essential for a working diagnosis, which is then confirmed by conjunctival biopsy. 83% of cases of CP are correctly diagnosed with conjunctival biopsy. The remaining 17% have a negative biopsy but clinical characteristics are so typical of CP that the clinician proceeds with therapy despite the negative biopsy. Certain eye infections, trauma, topical eye medications taken for some other purpose, and systemic diseases, e.g. sarcoidosis, can also cause conjunctival scarring. Hence, the treating physician may need to perform blood tests and other diagnostic tests, in addition to the conjunctival biopsy to reach a diagnosis.


The 'prognosis' of Ocular cicatricial pemphigoid usually refers to the likely outcome of Ocular cicatricial pemphigoid. The prognosis of Ocular cicatricial pemphigoid may include the duration of Ocular cicatricial pemphigoid, chances of complications of Ocular cicatricial pemphigoid, probable outcomes, prospects for recovery, recovery period for Ocular cicatricial pemphigoid, survival rates, death rates, and other outcome possibilities in the overall prognosis of Ocular cicatricial pemphigoid. Naturally, such forecast issues are by their nature unpredictable.


CP requires systemic immunosuppressive/immunoregulatory therapy. The medications suppress the patient's "rebellious" white blood cells to stop the self-destruction of body tissues. They are more effective and much safer than previously used systemic corticosteroids, if prescribed by a chemotherapy expert. Eye drops alone are not beneficial. Symptomatic treatment is also usually required for complications of CP. Dry eyes are treated with ointment lubricants, artificial tears and tear conservation measures. Lid hygiene and oral antibiotics are recommended for lid infection and inflammation. Removal of ingrowing eyelashes is essential to prevent them from damaging the cornea. Other measures such as surgical correction of eyelid deformities, are more successful when the disease has been under control for an extended time.