This 65 year-old man had had a five-year history of recurrent bilateral conjunctivitis. He is now registered blind and has the above appearance.
a. What is the diagnosis?Ocular cicatricial pemphigoid.
Figure 1 shows right ptosis (due to adhesion between the tarsal conjunctiva and bulbar conjunctiva and cornea) and misdirection of the right upper eyelashes. Figure 2 shows scarring of the cornea with neovascularization, symblepharon, scarring and shallowing of the inferior fornix.
b. What are the causes of blindness in this condition?Ocular cicatricial pemphigoid causes blindness through:
- Severe dry eyes. This is caused by the destruction of the goblet cells and accessory lacrimal glands through repeated inflammation. This can lead to keratitis and corneal keratinization.
- Scarring of the conjunctiva leads to cicatricial entropion and trichiasis causing corneal scarring.
c. What treatment may slow the progression of this condition?Steroid is useful in reducing the acute inflammation in the short term but does not appear to affect the progression of the disease. Dapsone is used in conjunction as a long term treatment as it can slow the progression. If the response to dapsone is limited, then immunosuppressive drugs such as methorexate, azathioprine or cylocphosphamide can be used.
d. Which parts of the body may also be involved in this condition?Ocular cicatricial pemphigoid is a systemic disease affecting the mucous membrane and the skin. In addition to the eye, inflammation and scarring of the mucous membrane can occur in the oesophagus, trachea, pharynx, vagina and urethra. Involvement can occur but does not lead to scarring.