Hereditary crystalline stromal dystrophy of Schnyder. II. Histopathology and ultrastructure.

Abstract

Although first described almost half a century ago (van Went and Wibaut, 1924) and firmly established as a distinct entity by Schnyder (I 929), proof that the crystalline deposits in this disorder are cholesterol was not forthcoming until quite recently (Delleman and Winkelman, I 968). Nearly 40 years ago Bonnet, Paufique, and Bonamour (I 934) demonstrated the presence of crystals which were birefringent and soluble in ether, and Sedan and Valles (I946) carried out specific histochemical staining for cholesterol, but in neither of these reports was the hereditary nature of the lesion established. Reports of bona fide cases of hereditary crystalline dystrophy examined histologically are scarce (Malbran, Paunessa, and Vidal, 1953; Paufique, Ravault, Bonnet, and Laurent, I964; Delleman and Winkelman, I968) and, apart from the case described by Offret, Payrau, Pouliquen, Faure, and Bisson (i 966), which must be accepted with some reservation since cholesterol crystals were not demonstrated, there is no account of the ultrastructural appearances in this condition.