| Identifier |
walsh_1999_s4_c2 |
| Title |
Multi-System Mystery |
| Creator |
Robert A. Egan, MD; Simmons Lessell, MD |
| Affiliation |
(RE) (SL) Boston, MA |
| Subject |
Malignant Atrophic Papulosis; Basal Ganglia Cerebrovascular Disease; Pupil Disorders; Cataract |
| History |
A 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. |
| Pathology |
Cortical infarcts and hemorrhages in the frontal lobe; Perivascular lymphocytiuc infiltration of skin along with annular deposition of mucin |
| Disease/Diagnosis |
Degos Disease |
| Clinical |
VA: 16/13- OD, 16/20 OS; Amsler grid test revealed haziness just below the midline OS; Tonometry: 18 OD, 15 OS; RAPD OS |
| Presenting Symptom |
Constricted visual fields OU |
| Neuroimaging |
MRI |
| Date |
1999 |
| References |
1. 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. |
| Language |
eng |
| Format |
application/pdf |
| Relation is Part of |
Case presented at the NANOS 1999 Walsh Session, February 6-7. |
| Collection |
Neuro-Ophthalmology Virtual Education Library: Walsh Session Annual Meeting Archives: https://novel.utah.edu/Walsh/ |
| Publisher |
North American Neuro-Ophthalmology Society |
| Holding Institution |
Spencer S. Eccles Health Sciences Library, University of Utah |
| Rights Management |
Copyright 2008. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
| ARK |
ark:/87278/s6cc13f5 |
| Setname |
ehsl_novel_fbw |
| ID |
178110 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s6cc13f5 |
