Acquired corneal dystrophy; dysgenesis of the anterior segment of the eye, blue scleral band, oligodontia, and metacarpal dysplasia (dysplasia oculo-dento-digitalis?).

RIEGER'S dysgenesis mesodermalis iridis et corneae, caused by a faulty differentiation in the anterior segment, is not limited to the mesodermal structures of the eye, but demonstrates a range of ectodermal anomalies as well. This is why Hagedoorn (1937) suggested the term "dysgenesis mesostromalis", stressing the frequent occurrence of ectodermal developmental anomalies of the iris and lens, combined with skeletal and dental anomalies. Dysplasia oculo-dento-digitalis is a syndrome in which, apart from signs of dysgenesis of the anterior segment of the eye, dental and skeletal anomalies are found together with campodactyly and syndactyly. Whether the two syndromes are related is as yet not at all certain, though Frandsen (1964) recently reported a case which he ascribed to each of the two syndromes. The present paper describes the case of a 16-year-old girl (Fig. 1) suffering from an acute corneal oedema due to a spontaneous (?) descemetolysis in which the question of the underlying developmental anomalies is still not completely cleared.