ANTERIOR CORNEAL-DYSTROPHY WITH DYSCOLLAGENOSIS (REIS-BUCKLERS TYPE)

Anterior corneal dystrophies involving Bowman's membrane and anterior stroma include several subtypes of uncertain etiology: the Reis-Buckle r, Thiel-Behnke, Grayson-Wilbrandt, and ''honeycomb'' dystrophies. The clinical and pathologic features of these dystrophies overlap to such a degree that they may represent variations of the same entity. Typic ally, they all present symptoms beginning in childhood, have a dominan t pattern of heredity, and manifest painful, recurrent corneal erosion s. Some cases have also been interpreted as representing macular and g ranular dystrophy. In the present report, we describe a pedigree of af fected patients whose corneal dystrophy shared many of the clinical an d pathologic features of the Reis-Bucklers and allied subtypes but whi ch differ from all in causing visual symptoms late in life and with mi nimal signs of recurrent erosion. Histopathology revealed a thickening of the anterior stroma by the addition of a partially disorganized an d degenerating tissue in which collagen Type III (fetal or repair coll agen) is intermixed with the normal (mature) Type I collagen in the en tire stroma. This is accompanied by irregular swelling of the basal ep ithelial cells and hyperplasia of the basement membrane (collagen Type IV, laminin and fibronectin), suggesting an aberrant influence of the epithelium on collagen synthesis.