A Case of Degos Disease Complicated By Constrictive Pericarditis In Remote Phase

Abstract Background: Degos disease, also known as malignant atrophic papulosis, is characterized by cutaneous manifestations due to chronic thrombo-obliterative vasculopathy. There have been reports of rare late-onset Degos disease complicated by constrictive pericarditis (CP). We report a case of CP caused by Degos disease that developed 20 years after diagnosis.Case presentation: A 62-year-old woman who has been taking aspirin for 20 years for Degos disease was hospitalized for worsening heart failure. The patient was diagnosed with CP and underwent pericardiectomy. Pathological findings suggested the involvement of Degos disease. The postoperative course was uneventful, and her heart failure and Degos disease did not worsen.Conclusions: This report suggests that Degos disease can cause long-term CP. Aspirin effectively inhibited the progression of Degos disease, and surgical treatment is necessary when heart failure due to CP is refractory to treatment.