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June 1963

The Ocular Manifestations of Hereditary Dystopic Lipidosis(Angiokeratoma Corporis Diffusum Universale)

Arch Ophthalmol. 1963;69(6):708-716. doi:10.1001/archopht.1963.00960040714005

Introduction  Angiokeratoma corporis diffusion universale was first reported as a dermatological entity.1,2 The term hereditary dystopic lipidosis was recently proposed by Rahman and co-workers3 to denote the systemic nature of the disease. The pathology of the disease has been well recognized by a number of investigators.4,5 A recent publication defines the disease, elucidates its inheritance, and illustrates many of its clinical and pathogical features.3 Like many other inborn errors of lipid metabolism, the disease begins early in life. Unlike other lipidosis the cellular storage is unique in distribution. The lipid accumulates in the myocardium, smooth muscles of the blood vessel walls, epithelial cells of kidney and cornea, and neurons of the myenteric plexuses and sympathetic ganglia. In the central nervous system the intermediolateral cell columns of the thoracic cord, dorsal autonomic nuclei of vagi, supraoptic, paraventricular and preoptic nuclei of hypothalamus, amygdala and substantia nigra are