Microangiopathy and retinal dystrophy

Two cases are described of an association of retinal dystrophy and a vasculopathy similar to Coat's disease. The first case was a female adult in whom the two sets of symptoms appeared almost simultaneously. In the second case the disease appeared in infancy, evolved rapidly during adolescence and led to blindness. In both cases, the vasculopathy developed over several years and then stabilised; in contrast, the dystrophy, which was of the paucipigmentary type, continued to get worse. From these two cases, and others described in the literature, it appears that many causes can be involved in the association of the two syndromes. A genetic factor that has been demonstrated in several families was not present in these two patients; there appears to have been an immunological or inflammatory process, not yet identified, that became active at the time of the rapid evolution of the dystrophy.