Affordable Access

Lethal systemic Degos disease with prominent cardio-pulmonary involvement.

Authors
  • Notash, Ali Y
  • Mazoochy, Hamed
  • Mirshams, Mostafa
  • Nikoo, Azita
Type
Published Article
Journal
Saudi medical journal
Publication Date
Jan 01, 2008
Volume
29
Issue
1
Pages
133–137
Identifiers
PMID: 18176689
Source
Medline
License
Unknown

Abstract

Degos disease DD is a rare obstructive vasculopathy characterized by distinctive skin lesions. Involvement of the soles, palms and genitalia is rare. In most cases, disease has an unfavorable course and involves gastrointestinal tract, central nervous system and occasionally other organs. Pleural and pericardial involvements are usually minor manifestations with prolonged course. Death occurs in approximately 50% of the patients usually due to intestinal perforation or central nervous system bleeding. We describe a 48-year-old man of lethal systemic DD. Widespread skin lesions with involvement of palmoplantar surfaces, genitalia and scalp were ignored for 3 years, whereas the disease revealed own malignant nature. The disorder progressed to nervous, gastrointestinal and cardiopulmonary system that led to death after 5 months from onset of systemic involvement as severe restrictive cardio-pulmonary insufficiency. Autopsy showed diffuse fibrotic changes in serosal membranes and internal organs.

Report this publication

Statistics

Seen <100 times