Article Text

Download PDFPDF
Case report
Chronic pleuritis leading to severe pulmonary restriction: a rare complication of Degos disease
  1. Biplab Kumar Saha and
  2. Scott Beegle
  1. Pulmonary and Critical Care Medicine, Albany Medical Center Hospital, Albany, New York, USA
  1. Correspondence to Dr Biplab Kumar Saha; spanophiliac{at}


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.

  • respiratory medicine
  • rheumatology
  • gastrointestinal surgery
  • pericardial disease
  • skin

Statistics from

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.


  • Contributors BKS and SB were directly involved in patient care. BKS planned, collected data and prepared the initial manuscript. SB supervised the preparation of the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Next of kin consent obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.