Ophthalmic changes of Degos' disease (malignant atrophic papulosis)

A 49-year-old woman with typical skin lesions of Degos' disease was found to have an afferent pupillary defect, and altitudinal field loss. This loss was probably secondary to vascular occlusion in the optic nerve of a patient with a congenital anomalous retinal vascular pattern. The patient has had no other systemic involvement after a follow-up of 4 years. Three other patients seen in the past had eye involvement and involvement of the central nervous system and died between 1 and 16 years of the diagnosis. Multiple ocular manifestations of Degos' disease have been reported affecting the eyelids, bulbar conjunctivae, retina, and choroid in varying degrees. Diplopia, visual field defects, ophthalmoplegia, ptosis, papilledema, and optic atrophy are ocular changes secondary to involvement of the central nervous system.