{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,28]],"date-time":"2026-08-28T22:46:01Z","timestamp":1787957161820,"version":"build-2784847793"},"reference-count":82,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"1","content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2022,1]]},"abstract":"<jats:sec>\n                    <jats:title\/>\n                    <jats:p>Malignant atrophic papulosis (MAP), or systemic Degos disease, is an obliterative vasculopathy of unknown origin, characterized by erythematous papules found on the skin, central nervous system (Neuro-MAP) and gastrointestinal tract. Neurological involvement occurs in approximately 20% of systemic cases, is progressive and largely fatal. It can be described in two forms: 1) the parenchymal presenting with meningoencephalitis and meningomyelitis and 2) the neurovascular presenting with large cerebral infarcts, intracranial and subarachnoid hemorrhage, subdural hematoma and venous sinus thrombosis. Predilection to subdural hematoma or hygroma is characteristic for neurological involvement in MAP in comparison to other vasculpathies and vasculitides. Peripheral nervous system manifestations are less common and include polyradiculopathy, neuropathy, and myopathy. CSF analysis usually shows mild to moderate pleocytosis, increased protein content, and normal glucose. Brain MRI may reveal cortical, subcortical and deep white matter ischemic lesions with possible nodular, leptomeningeal, dural, or ependymal enhancement. Spinal cord MRI may reveal patchy lesions from the periphery to the center or cord atrophy in progressive course. Neurological involvement in MAP has a grave prognosis. The interval from onset of papulosis to death averages two years in patients with neurological involvement. There is no confirmed treatment for MAP but there are promising reports with eculizumab and treprostinil.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.4103\/0028-3886.338719","type":"journal-article","created":{"date-parts":[[2024,2,21]],"date-time":"2024-02-21T00:38:10Z","timestamp":1708475890000},"page":"5-10","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":6,"title":["Neurological Involvement in Malignant Atrophic Papulosis"],"prefix":"10.4103","volume":"70","author":[{"given":"Samaneh","family":"Yousefi","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Afshin","family":"Borhani-Haghighi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anahid","family":"Safari","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lee","family":"Shapiro","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"R1-3-20240907","doi-asserted-by":"crossref","first-page":"783","DOI":"10.1007\/BF01828450","article-title":"Multiple Hautnekrosen bei Thromboangitis Obliterans","volume":"181","author":"K\u00f6hlmeier","year":"1941","journal-title":"Arch Dermatol Syphil"},{"key":"R2-3-20240907","first-page":"148","article-title":"Dermatite papulo-squameuse atrophiante","volume":"49","author":"Degos","year":"1942","journal-title":"Bull Soc Fr Dermatol Syphiligr"},{"key":"R3-3-20240907","first-page":"646","article-title":"Nervous system involvement in Degos disease","volume":"26","author":"Amato","year":"2005","journal-title":"AJNR"},{"key":"R4-3-20240907","doi-asserted-by":"crossref","first-page":"483","DOI":"10.1111\/j.1365-2230.2007.02497.x","article-title":"Malignant atrophic papulosis","volume":"32","author":"Scheinfeld","year":"2007","journal-title":"Clin Exp Dermatol"},{"key":"R5-3-20240907","doi-asserted-by":"crossref","first-page":"18","DOI":"10.1111\/j.1525-1470.2007.00326.x","article-title":"Benign cutaneous Degos disease in a 16-year-old girl","volume":"24","author":"Wilson","year":"2007","journal-title":"Pediatr Dermatol"},{"key":"R6-3-20240907","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1046\/j.1468-3083.2001.00216.x","article-title":"Malignant atrophic papulosis (Degos' disease): clinicopathological correlations","volume":"15","author":"Chave","year":"2001","journal-title":"J Eur Acad Dermatol Venereol"},{"key":"R7-3-20240907","doi-asserted-by":"crossref","first-page":"56","DOI":"10.1097\/00007611-196201000-00014","article-title":"Degos' disease (papulosis atrophicans maligna): report of a case with degenerative disease of the central nervous system","volume":"55","author":"Gever","year":"1962","journal-title":"South Med J"},{"key":"R8-3-20240907","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1177\/088307388800300110","article-title":"Childhood Degos disease with prominent neurological symptoms: report of a clinicopathological case","volume":"3","author":"Rosemberg","year":"1988","journal-title":"J Child Neurol"},{"key":"R9-3-20240907","doi-asserted-by":"crossref","first-page":"469","DOI":"10.1111\/j.1365-2133.1984.tb06611.x","article-title":"Six cases of malignant atrophic papulosis (Degos' disease) occurring in one family","volume":"111","author":"Kisch","year":"1984","journal-title":"Br J Dermatol"},{"key":"R10-3-20240907","doi-asserted-by":"crossref","first-page":"262","DOI":"10.1001\/archopht.1968.03850040264007","article-title":"Malignant atrophic papulosis. 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