It should be remembered that patients might also suffer with other eye problems not associated with EB, such as cataracts and refractive errors (e.g. short sightedness requiring glasses correction). These are best dealt with separately, although the patient should ensure the clinician is aware they have EB.

Examination

One of the anxieties encountered by EB patients is having the eye examined. Very often a good history from the patient can point to a diagnosis, so the patient is advised to have information available, such as what has happened (sudden pain and watering of the eye), when (this morning on waking), past history (happened three times before in same eye) etc. It may help to write it down, and some patients even keep a diary of events if they have re-occurring eye problems.

There are several simple rules to follow when examining the eyes of a patient with EB. Most ophthalmologists are very skilled in the care of patients with problems such as EB, but if the patient is in doubt, the following advice may prove helpful.

If at all possible a no touch technique should be used. In the adult patient this is easier since the patient is often able to assist the clinician. In the case of a child, distraction with toys or lights can be useful to promote opening of the eyes.

Application of a local anaesthetic drop such as Proxymetacaine Hydrochloride, which does not sting on application as much as say Benoxinate Hydrochloride, may be useful if the patient has pain causing them to be unable to open the affected eye.

The most important factor is that the lids should not be forced open, since this can result in further trauma to either the lids themselves or the conjunctiva/cornea. The lids can be very gently moved to examine areas of the conjunctiva not in view when the patient has their eyes open, but again touch should be very light, and if the patient is not able to co-operate, it is best to discontinue.

Examination by torch or slit lamp (if available), should be done obliquely, since shining the light directly into the eye causes spasm of the iris and resultant pain, photophobia, and closure of the eye.

Instilling a drop of Fluorescein (also available combined with Proxymetacaine, requiring only one drop application for anaesthesia and examination), will show any epithelial defects that may not be visible on simple light examination.