[Clinical and ultrastructural classification of Reis-Bückler corneal dystrophy]

Reis-Bücklers' corneal dystrophy has been incorrectly defined in the English literature: the corneal opacities described in most reports on it do not correspond to Bücklers' original findings, but are equivalent to Thiel and Behnke's honeycomb corneal dystrophy. Moreover, the synonym "annular dystrophy" is based on a misunderstanding and ought to be replaced by the term "maplike dystrophy". Perhaps due to the misnomer, annular or honeycomblike subepithelial opacities have come to be regarded as Reis-Bücklers' dystrophy. Subsequently, histologic and ultra-structural features were also evolved from such supposed cases, and the curly, electron-dense filaments were regarded as pathognomonic. The erroneous definition in the standard literature has been causing diagnostic confusion ever since. Research in the older literature and studies of the author's own patients have established the following original features of Reis-Bücklers' dystrophy: (1) dominant inheritance, (2) early manifestation and rapid progression, (3) painful attacks during childhood, (4) subepithelial corneal opacities extending almost to the limbus, (5) maplike opacity pattern, (6) sheetlike deposits replacing Bowman's membrane in histologic sections, (7) electron-dense rod-shaped bodies observed by electron microscopy. The original Reis-Bücklers' dystrophy resembles granular corneal dystrophy (Groenouw I) histochemically and ultrastructurally, but differs from it in its clinical symptoms, corneal opacity pattern, histopathological arrangement, and probably gene linkage as well. The condition commonly referred to as Reis-Bücklers' dystrophy in the literature is in fact Thiel and Behnke's honeycomb corneal dystrophy.