Intrathoracic manifestations of Degos' disease (malignant atrophic papulosis)

Abstract

Degos' disease (malignant atrophic papulosis) is a rare multisystemic disease with characteristic cutaneous lesions, abdominal symptoms and often rapidly fatal course. Our review of the reported intrathoracic involvement in the more than 60 reported cases of Degos' disease suggests that pleuritis and pericarditis are manifestations of the underlying pathophysiologic process. Seventeen of these reported cases were said to have intrathoracic lesions, which were, in most cases, incidental findings at autopsy. We present an illustrative report of a case in which chronic pleuritis and pericarditis were the major causes of morbidity in a 32-year-old woman with Degos' disease.