Keratouveitis--two families with a dominantly inherited disorder

Herein we describe a relapsing acute keratouveitis without known aetiology. The disorder has been found in two families and shows an autosomal dominant inheritance. Acute anterior uveitis can be traced for five generations in one of these families. In the same family there is also an association to an autosomal dominantly inherited vascular disorder (Osler-Rendu-Weber disease). The anterior uveitis has an acute onset, a recurrent pattern and a mild activity. The associated keratitis is seen as a midstromal thin flat disc in the central cornea, sometimes with folds in Descemet's membrane as a sign of oedema. This causes a change in refraction and a mild reduction of the corrected visual acuity. There is a prompt response to topical steroid treatment, but the corneal changes have in one case become permanent. Clinical documentation and the mode of inheritance is presented.