{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,31]],"date-time":"2026-07-31T02:01:48Z","timestamp":1785463308493,"version":"3.56.0"},"reference-count":21,"publisher":"BMJ","issue":"12","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2019,11,15]]},"published-print":{"date-parts":[[2019,12]]},"abstract":"<jats:p>This case demonstrates chronic fibrosing pleuritis, as a rare pulmonary aetiology for mortality in patients with Degos disease or malignant atrophic papulosis (MAP). Knowledge of this unusual complication will help physicians identify this entity early and provide appropriate treatment.<\/jats:p>\n               <jats:p>Patients with MAP die from gastrointestinal and brain involvement within 2\u20133 years of diagnosis. This case is unique as the patient survived for 9\u2009years and died secondary to respiratory failure, which had not been reported before. Our patient was a young man, diagnosed with MAP at the age of 17. His skin and gastrointestinal disease were controlled with eculizumab and parenteral treprostinil. The patient developed severe restrictive pulmonary disease, required ventilatory support, and died from respiratory failure. An autopsy revealed chronic fibrosis pleuritis. Longer surviving patients with MAP might suffer from significant respiratory disease. Pulmonary function test should be obtained to identify subclinical respiratory limitation.<\/jats:p>","DOI":"10.1136\/bcr-2019-232759","type":"journal-article","created":{"date-parts":[[2019,12,9]],"date-time":"2019-12-09T04:46:58Z","timestamp":1575866818000},"page":"e232759","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":5,"title":["Chronic pleuritis leading to severe pulmonary restriction: a rare complication of Degos disease"],"prefix":"10.1136","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3595-8804","authenticated-orcid":false,"given":"Biplab Kumar","family":"Saha","sequence":"first","affiliation":[{"name":"Pulmonary and Critical Care Medicine, Albany Medical Center Hospital, Albany, New York, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Scott","family":"Beegle","sequence":"additional","affiliation":[{"name":"Pulmonary and Critical Care Medicine, Albany Medical Center Hospital, Albany, New York, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2019,12,8]]},"reference":[{"key":"2025082700182382000_12.12.e232759.1","doi-asserted-by":"crossref","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"},{"key":"2025082700182382000_12.12.e232759.2","doi-asserted-by":"crossref","first-page":"783","DOI":"10.1007\/BF01828450","article-title":"Multiple Hautnekrosen bei Thrombangiitis obliterans","volume":"181","author":"K\u00f6hlmeier","year":"1941","journal-title":"Arch f Dermat"},{"key":"2025082700182382000_12.12.e232759.3","first-page":"148","article-title":"Dermatite papulosqameuse atrophiante","volume":"49","author":"Degos","year":"1942","journal-title":"Bull Soc Fr Derm Syph"},{"key":"2025082700182382000_12.12.e232759.4","first-page":"480","article-title":"[Malignant atrophic papulosis (K\u00f6hlmeier-Degos disease)]","volume":"40","author":"Burg","year":"1989","journal-title":"Hautarzt"},{"key":"2025082700182382000_12.12.e232759.5","first-page":"280","article-title":"[Ultrastructural study of the Dego's disease (report of a new case and literature review) (author's transl)]","volume":"104","author":"Olmos","year":"1977","journal-title":"Ann Dermatol Venereol"},{"key":"2025082700182382000_12.12.e232759.6","first-page":"131","article-title":"Clinical and histologic findings in Degos' syndrome (malignant atrophic papulosis)","volume":"35","author":"Su","year":"1985","journal-title":"Cutis"},{"key":"2025082700182382000_12.12.e232759.7","doi-asserted-by":"crossref","first-page":"90","DOI":"10.1159\/000247999","article-title":"Malignant atrophic papulosis: response to antiplatelet therapy","volume":"180","author":"Drucker","year":"1990","journal-title":"Dermatology"},{"key":"2025082700182382000_12.12.e232759.8","doi-asserted-by":"crossref","DOI":"10.1055\/s-0038-1660113","article-title":"Fibrinolysis in Degos' disease","volume":"54","author":"P\u00e1ramo","year":"1985","journal-title":"Thromb Haemost"},{"key":"2025082700182382000_12.12.e232759.9","doi-asserted-by":"crossref","first-page":"365","DOI":"10.1016\/S0190-9622(86)70180-1","article-title":"Malignant atrophic papulosis: absence of circulating immune complexes or vasculitis","volume":"15","author":"Tribble","year":"1986","journal-title":"J Am Acad Dermatol"},{"key":"2025082700182382000_12.12.e232759.10","doi-asserted-by":"publisher","DOI":"10.1309\/AJCP66QIMFARLZKI"},{"key":"2025082700182382000_12.12.e232759.11","doi-asserted-by":"crossref","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"},{"key":"2025082700182382000_12.12.e232759.12","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1159\/000248781","article-title":"Skin lesion resembling malignant atrophic papulosis in lupus erythematosus","volume":"175","author":"Doutre","year":"1987","journal-title":"Dermatology"},{"key":"2025082700182382000_12.12.e232759.13","doi-asserted-by":"publisher","DOI":"10.1016\/S0190-9622(97)80407-0"},{"key":"2025082700182382000_12.12.e232759.14","first-page":"260","article-title":"Malignant atrophic papulosis. Report of a case with multiple ophthalmic findings","volume":"51","author":"Thomas","year":"2003","journal-title":"Indian J Ophthalmol"},{"key":"2025082700182382000_12.12.e232759.15","doi-asserted-by":"crossref","first-page":"493","DOI":"10.1111\/j.1365-4632.2008.03394.x","article-title":"A case of Degos disease with pleuropericardial fibrosis, jejunal perforation, hemiparesis, and widespread cutaneous lesions","volume":"47","author":"Shahshahani","year":"2008","journal-title":"Int J Dermatol"},{"key":"2025082700182382000_12.12.e232759.16","first-page":"335","article-title":"Fibrothorax and severe lung restriction secondary to lupus pleuritis and its successful treatment by pleurectomy","volume":"9","author":"Sharma","year":"2002","journal-title":"Can Respir J"},{"key":"2025082700182382000_12.12.e232759.17","doi-asserted-by":"crossref","first-page":"361","DOI":"10.1016\/j.arbres.2011.02.010","article-title":"Systemic diseases and the pleura","volume":"47","author":"Ferreiro","year":"2011","journal-title":"Archivos de Bronconeumolog\u00eda"},{"key":"2025082700182382000_12.12.e232759.18","first-page":"421","article-title":"A case of systemic malignant atrophic papulosis (K\u00f6hlmeier-Degos' disease)","volume":"97","author":"Fern\u00e1ndez-P\u00e9rez","year":"2005","journal-title":"J Natl Med Assoc"},{"key":"2025082700182382000_12.12.e232759.19","doi-asserted-by":"crossref","first-page":"84","DOI":"10.1046\/j.1365-2559.1996.t01-1-258289.x","article-title":"Malignant atrophic papulosis (Degos' disease) with extensive cardiopulmonary involvement","volume":"28","author":"Mauad","year":"1996","journal-title":"Histopathology"},{"key":"2025082700182382000_12.12.e232759.20","doi-asserted-by":"crossref","unstructured":"Polito J , Toledo A , Shapiro L . Early detection of systemic Degos disease (DD) or malignant atrophic papulosis (MAP) may increase survival (Abstract). 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