Elastica Disease

PSEUDOXANTHOMA elasticum is a well-known disease, described and named by Darier in 1896.* When associated with retinal alterations (angioid streaks, etc.), the complex is known as the Grönblad-Strandberg syndrome. The disease is presumed to be due, basically, to elastic tissue degeneration. Cutaneous lesions vary from pinhead- to pea-sized yellowish flat papules that often fuse to form plaques. The symmetrical distribution is in flexural folds (axillae, antecubital fossae, inguinal regions), the sides of the neck, upper and inner aspects of the thighs, and over the abdomen. In extreme cases there is obvious loss of cutaneous elasticity. The unilateral or bilateral retinal changes, accompanied by loss of visual acuity, appear to be due to degenerative changes of the elastica of Bruch's membrane. The disease may be found at any age in both sexes, but most commonly it begins in childhood or early adult life. A familial history, fairly regularly elicited, emphasizes the