Background: Degos’ disease or malignant atrophic papulosis, is a rare obstructive vasculopathy of unknown origin, characterized by distinctive skin lesions, gastrointestinal tract and central nervous system involvement. Constrictive pericarditis has also been reported and is considered to be related to the vascular process of the disease.
Case Report: We report the case of a 42-year-old woman with undiagnosed Degos’ disease who developed acute pericarditis. To the best of our knowledge this is the first report of acute pericarditis associated with malignant atrophic papulosis.
Conclusions: Clinicians should bear in mind that acute pericarditis may develop during the course of Degos’ disease and arrange appropriate clinical and laboratory examinations in cases of relevant symptomatology.

Keywords: Acute pericarditis, malignant atrophic papulosis, Degos’ disease