{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,3]],"date-time":"2026-06-03T07:25:47Z","timestamp":1780471547274,"version":"3.54.1"},"reference-count":15,"publisher":"American Academy of Pediatrics (AAP)","issue":"Supplement_5","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2018,4,1]]},"abstract":"<jats:p>Malignant atrophic papulosis (MAP), also known as Degos disease, is an extremely rare disease that is characterized by its unique skin presentation (namely, central, porcelain-white atrophic lesions with a telangiectatic rim). MAP has the following 2 variants: cutaneous MAP is manifested in the skin alone, whereas systemic MAP affects the gastrointestinal tract, central nervous system, lungs, and other internal organs. Some patients who presented with only cutaneous symptoms at first may develop systemic symptoms several years later. Although the exact pathologic mechanisms are unclear, Magro et al suggested in a recent study that MAP is a vascular injury syndrome that involves complement component C5b\u20139 complex deposition and high expression of interferon-\u03b1. The prognosis of systemic MAP is poor and typically fatal within a few years. Nonetheless, because the C5b\u20139 complex is detected in MAP, some researchers have suggested combined treatment with eculizumab (a humanized monoclonal antibody against C5) and treprostinil (a prostacyclin analog). Here, we report on a girl with systemic MAP who had severe central nervous system involvement and responded to eculizumab.<\/jats:p>","DOI":"10.1542\/peds.2016-4206","type":"journal-article","created":{"date-parts":[[2018,4,2]],"date-time":"2018-04-02T08:35:25Z","timestamp":1522658125000},"page":"S481-S484","source":"Crossref","is-referenced-by-count":14,"title":["Pediatric Malignant Atrophic Papulosis"],"prefix":"10.1542","volume":"141","author":[{"given":"Yung-Chieh","family":"Huang","sequence":"first","affiliation":[{"name":"Departments of Pediatrics and"},{"name":"Division of Pediatrics, Puli Branch, Taichung Veterans General Hospital, Nantou, Taiwan; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jiaan-Der","family":"Wang","sequence":"additional","affiliation":[{"name":"Departments of Pediatrics and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fang-Yi","family":"Lee","sequence":"additional","affiliation":[{"name":"Pathology and Laboratory Medicine, Taichung Veterans General Hospital, Taichung, Taiwan;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lin-Shien","family":"Fu","sequence":"additional","affiliation":[{"name":"Departments of Pediatrics and"},{"name":"Department of Pediatrics, National Yang-Ming University, Taipei, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"417","published-online":{"date-parts":[[2018,4,1]]},"reference":[{"issue":"1","key":"2021121011392820800_B1","doi-asserted-by":"crossref","first-page":"110","DOI":"10.1111\/bjd.12642","article-title":"Malignant and benign forms of atrophic papulosis (K\u00f6hlmeier-Degos disease): systemic involvement determines the prognosis.","volume":"170","author":"Theodoridis","year":"2014","journal-title":"Br J Dermatol"},{"issue":"3","key":"2021121011392820800_B2","doi-asserted-by":"crossref","first-page":"302","DOI":"10.1111\/j.1525-1470.2010.01287.x","article-title":"Malignant atrophic papulosis: a case report with severe visual and neurological impairment.","volume":"28","author":"Guti\u00e9rrez-Pascual","year":"2011","journal-title":"Pediatr Dermatol"},{"issue":"4","key":"2021121011392820800_B3","doi-asserted-by":"crossref","first-page":"550","DOI":"10.1177\/0883073812474692","article-title":"Severe neurologic involvement of Degos disease in a pediatric patient.","volume":"29","author":"Karao\u011flu","year":"2014","journal-title":"J Child Neurol"},{"issue":"1","key":"2021121011392820800_B4","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":"2021121011392820800_B5","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1186\/1750-1172-8-52","article-title":"Effective treatment of malignant atrophic papulosis (K\u00f6hlmeier-Degos disease) with treprostinil\u2013early experience.","volume":"8","author":"Shapiro","year":"2013","journal-title":"Orphanet J Rare Dis"},{"issue":"3","key":"2021121011392820800_B6","doi-asserted-by":"crossref","first-page":"636","DOI":"10.1212\/WNL.46.3.636","article-title":"Skin lesion with a fatal neurologic outcome (Degos\u2019 disease).","volume":"46","author":"Subbiah","year":"1996","journal-title":"Neurology"},{"issue":"5, pt 1","key":"2021121011392820800_B7","doi-asserted-by":"crossref","first-page":"831","DOI":"10.1016\/0190-9622(95)91843-4","article-title":"Absence of antiphospholipid and anti-endothelial cell antibodies in malignant atrophic papulosis: a study of 15 cases.","volume":"33","author":"Assier","year":"1995","journal-title":"J Am Acad Dermatol"},{"issue":"24","key":"2021121011392820800_B8","doi-asserted-by":"crossref","first-page":"2327","DOI":"10.1056\/NEJMcpc1304161","article-title":"Case records of the Massachusetts General Hospital. Case 18-2014. A 32-year-old man with a rash, myalgia, and weakness.","volume":"370","author":"Burgin","year":"2014","journal-title":"N Engl J Med"},{"issue":"3A","key":"2021121011392820800_B9","doi-asserted-by":"crossref","first-page":"692","DOI":"10.1590\/S0004-282X2009000400022","article-title":"K\u00f6hlmeier-Degos disease (malignant atrophic papulosis) and neurologic involvement.","volume":"67","author":"Slaviero","year":"2009","journal-title":"Arq Neuropsiquiatr"},{"issue":"24","key":"2021121011392820800_B10","doi-asserted-by":"crossref","first-page":"2575","DOI":"10.1056\/NEJMcpc069029","article-title":"Case records of the Massachusetts General Hospital. Case 38-2006. A 5-year-old boy with headache and abdominal pain.","volume":"355","author":"Caviness","year":"2006","journal-title":"N Engl J Med"},{"issue":"6","key":"2021121011392820800_B11","doi-asserted-by":"crossref","first-page":"1274","DOI":"10.1016\/j.jaad.2016.09.015","article-title":"Diffuse atrophic papules and plaques, intermittent abdominal pain, paresthesias, and cardiac abnormalities in a 55-year-old woman.","volume":"75","author":"Oliver","year":"2016","journal-title":"J Am Acad Dermatol"},{"issue":"5","key":"2021121011392820800_B12","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":"2021121011392820800_B13","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1186\/1750-1172-8-10","article-title":"Malignant atrophic papulosis (K\u00f6hlmeier-Degos disease) - a review.","volume":"8","author":"Theodoridis","year":"2013","journal-title":"Orphanet J Rare Dis"},{"issue":"4","key":"2021121011392820800_B14","doi-asserted-by":"crossref","first-page":"599","DOI":"10.1309\/AJCP66QIMFARLZKI","article-title":"Degos disease: a C5b-9\/interferon-\u03b1-mediated endotheliopathy syndrome.","volume":"135","author":"Magro","year":"2011","journal-title":"Am J Clin Pathol"},{"key":"2021121011392820800_B15","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1186\/1750-1172-8-185","article-title":"The effects of Eculizumab on the pathology of malignant atrophic papulosis.","volume":"8","author":"Magro","year":"2013","journal-title":"Orphanet J Rare Dis"}],"container-title":["Pediatrics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/publications.aap.org\/pediatrics\/article-pdf\/141\/Supplement_5\/S481\/886588\/peds_20164206.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/publications.aap.org\/pediatrics\/article-pdf\/141\/Supplement_5\/S481\/886588\/peds_20164206.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,12,11]],"date-time":"2021-12-11T00:23:09Z","timestamp":1639182189000},"score":1,"resource":{"primary":{"URL":"https:\/\/publications.aap.org\/pediatrics\/article\/141\/Supplement_5\/S481\/34477\/Pediatric-Malignant-Atrophic-Papulosis"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,4,1]]},"references-count":15,"journal-issue":{"issue":"Supplement_5","published-online":{"date-parts":[[2018,4,1]]},"published-print":{"date-parts":[[2018,4,1]]}},"URL":"https:\/\/doi.org\/10.1542\/peds.2016-4206","relation":{},"ISSN":["0031-4005","1098-4275"],"issn-type":[{"value":"0031-4005","type":"print"},{"value":"1098-4275","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,4,1]]}}}