Gronblad-Strandberg Syndrome in Three Consecutive Brother and Sisters

ABSTRACT

Pseudoxanthoma elasticum is an inherited disease with degenerative changes in elastic tissue of human body. Skin lesion in pseudoxanthoma elasticum is easily understood and histopathologic finding of skin lesion is chracteristic in diagnosis of pseudoxanthoma elasticum. Pseudoxanthoma elasticum combined with the both dermatologic and ocular manifestations is called Gronblad-Strandberg syndrome. Angioid streak appears ophthalmoscopically as a network of red or dark brown streaks mainly around optic disc and may occur alone or in association with various systemic diseases involving pseudoxanthoma elasticum. Fluorescein angiography of fundus is evaluated as a means of detecting early angioid streak or some other ocular manifestations of pseudoxanthoma elasticum which may be observable only by this method and not previously described. To our knowledge, this is the first three cases of Gronblad-Strandberg syndrome in Korean consecutive siblings which are presented with pathologic and fluorescein angiographic studies.