Chronic pleuritis leading to severe pulmonary restriction: a rare complication of Degos disease. Issue 12 (8th December 2019)

• Record Type: Journal Article
• Authors: Saha, Biplab Kumar; Beegle, Scott
• Is Part Of: BMJ case reports. Volume 12:Issue 12(2019)
• Publication Date: 2019-12-08
• Subjects: respiratory medicine -- rheumatology -- gastrointestinal surgery -- pericardial disease -- skin; Medicine -- Case studies -- Periodicals; 610.5
• DOI: 10.1136/bcr-2019-232759
• Languages: English
• ISSNs: 1757-790X

Abstract

Abstract : 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. Patients with MAP die from gastrointestinal and brain involvement within 2–3 years of diagnosis. This case is unique as the patient survived for 9 years 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.