Multi-System Mystery

Constricted visual fields OUA 44-year old male with constricted visual fields, lingering on the left. Previous history significant for rash on hands, forearms and chest; cataract OS, atrial fibrillation, pericardial effusion and hypertension.VA: 16/13- OD, 16/20 OS; Amsler grid test revealed haziness just below the midline OS; Tonometry: 18 OD, 15 OS; RAPD OSMRICortical infarcts and hemorrhages in the frontal lobe; Perivascular lymphocytiuc infiltration of skin along with annular deposition of mucin1. Su WP, Schroeter AL, Lee DA, Hsu T, Muller SA. Clinical and histologic findings in Degos' syndrome (malignant atrophic papulosis). Cutis 35:131-138; 1985. 2. Subbiah P, Wij dicks E, Muenter M, Carter J, Connolly S. Skin lesion with a fatal neurologic outcome (Degos' disease). Neurology 46: 636-640; 1996. 3. Burrow IN, Blumbergs PC, Iyer PV, Hallpike JF. Kohlmeier-Degos disease: a multisystem vasculopathy with progressive cerebral infarction. AustNZJMed2\: 49-51; 1991. 4. Casparie MK, Meyer JW, van Huystee BE, Kneppelhout J, Mulder CJ. Endoscopic and histopathologic features of Degos' disease. Endoscopy 23: 231-233; 1991. 5. Vanderhaeghen JJ, Joffroy A, Achten G, et al. Lesions vasculaires dans la papulose atrophiante maligne de Degos: atteinte disseminee du systeme nerveux avec infiltration lipidique de l'intima des arteres sous-arachnoidiennes. Pathol Eur 3: 1-26; 1968. 6. Kalis B, Guillaine R, Fenzy A, et al. A propos d'un nouveau cas de papulose maligne de Degos. Bull Soc Fr Dermatol Syphiligr 75: 35-9; 1968. 7.Culicchia CF, Gol A, Erickson EE. Diffuse central nervous system involvement in papulosis atrophicans maligna. Neurology 12: 503-9; 1962. 8. Winkelmann RK, Howard FM Jr, Perry HO, Miller RH. Malignant papulosis of skin and cerebrum; a syndrome of vascular thrombosis: Arch Dermatol 87: 54-62; 1963. 9. Aizawa H, Takase Y, Inoue K, Murayama S, Mannen T. An autopsy case of Degos disease with neurological symptoms -neuropathological observations and increased platelet aggregation. Rinsho Shinkeigaku 32(1): 23-9; 1992. 10. McFarland HR, Wood WG, Drowns BV, Meneses ACO. Papulossi atrophicans maligna (Kohlmeier-Degos disease): A disseminated occlusive vasculopathy. Ann Neurol 3: 388-392; 1978