Malignant Atrophic Papulosis (Degos' Syndrome)

Eye in the skin- A review article

“…Ocular involvement in Degos' syndrome has included atrophic skin lesions of the eyelids, conjunctival microaneurysms and telangiectasias, atrophic, plaquelike lesions of the conjunctiva. 60 Atrophic chorioretinal scars are also noted. Episcleral, Optic disc, retinal, and choroidal vessels showed thickening of the walls, subendothelial fibrosis & fibrinoid necrosis.…”

Progressive Systemic Sclerosis With Malignant Atrophic Papulosis

“…This rare, usually fatal, cutaneovisceral syndrome was first described by Kohlmeier (1941)4 and Degos et al (1942). 6 Following the description of at least 29 cases in the European and North American literature68 since 1942, MAP is now con¬ ceived of as a truly systemic disease which may involve the brain,911 pericardium,9 my¬ ocardium,9 bladder,12 and kidney,9'12'13 conjunctivae and retina6'81114 as well as the skin and gastrointestinal tract. Yet the etiol-ogy is obscure.…”