Ocular Pemphigoid

Ocular pemphigoid has several other names such as ocular cicatricial pemphigoid, pemphigoid and benign mucous membrane pemphigoid.

It is an autoimmune condition in which the body produces chemicals called antibodies that damage itself. The reason that this process occurs is unknown - it is not caused by anything the patient has done and will not be cured or improved by any change in lifestyle or diet. There are many other autoimmune conditions, for example thyroid deficiency and rheumatoid arthritis.

The condition affects the eyes by causing inflammation of the conjunctiva which is the thin layer covering both the white part of the eye (the sclera) and the inner surface of the eyelids. The inflammation causes redness, discomfort, grittyness and dryness of the eyes. If the condition is not treated it may permanently damage sight by causing scarring of the cornea, the 'front window' of the eye.

Scarring of the conjunctiva in ocular pemphigoid
Note the white scar on the inner surface of the lower lid
(bottom arrow) and the two ridges (called symblephara)
which are also a form of conjunctival scar (top arrow)
(The lower lid is pulled down)

Symblephara (conjunctival scars) are important
clues to the diagnosis of ocular pemphigoid
(The eyelid has been pulled upwards to show them)

The natural course of ocular pemphigoid is for it to persist for many years with periods of spontaneous remission and flare-ups. Modern treatment has revolutionised the prognosis by reducing both the length of the disease and its severity. The treatment must be taken for a prolonged period and occasionally causes side-effects but has a good chance of success if used conscientiously.

Two types of treatment are necessary. Eye drops. These may include steroid drops to reduce inflammation and lubricants to keep the eyes moist. Tablets. These suppress the body's tendency to attack the eyes. Tablets are much more effective than eye drops in treating ocular pemphigoid. Depending on how advanced the condition is, I initially prescribe either dapsone or a combination of steroid and cyclophosphamide. Regular blood tests are needed to ensure that there are no side effects - I discuss this subject carefully with the patient before beginning treatment.

The treatment must be used until the disease has gone into remission and then for a suitable period longer. It can then be gradually reduced and eventually stopped.

Ocular pemphigoid can sometimes involve other parts of the body such as the mouth or skin in which case I look after the patient jointly with a dermatologist (skin specialist).