Since 1884, when Balzer first described pseudoxanthoma elasticum, periodic reports have appeared in the literature. In 1889 Chauffard studied such a case which he called "xanthelasma disseminatum et symmetricum." This case was further studied some seven years later by Darier, who made histologic examinations of the tissue for the first time and felt the disease was one of "elastorrhexis." This condition was originally felt to be a cutaneous disease. However, as more and more case reports crept into the literature, it became more obvious that this was a generalized systemic condition. This idea was first suggested by Hallopeau and Laffitte1in 1903 in the presentation of a case with associated visual impairment. They proposed the theory that the skin condition and chorioretinitis present were related. Subsequent investigations have proved beyond much doubt the truly systemic involvement in these cases The sites of predilection for the cutaneous appearance