Degos disease complicated by constrictive pericarditis in remote phase: a case report

Abstract

Background: Degos disease, also known as malignant atrophic papulosis, is characterised by cutaneous manifestations due to chronic thrombo-obliterative vasculopathy. There have been reports of the rare late-onset Degos disease complicated by constrictive pericarditis (CP). This study reports a case of CP caused by Degos disease that developed 20 years after diagnosis.

Case presentation: A 62-year-old woman who had been taking aspirin for 20 years for Degos disease was hospitalised for worsening of 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: The study findings suggests that Degos disease can cause long-term CP. Aspirin effectively inhibited the progression of Degos disease, and surgical treatment was necessary when heart failure due to CP was refractory to treatment.

Keywords: Constrictive pericarditis; Degos disease; Heart failure.

Fig. 1

Computed tomography demonstrating bilateral pleural effusion and pericardial effusion with marked calcification of the pericardium

Fig. 2

Cardiac catheterisation demonstrating equal right and left ventricular end-diastolic pressures and square root signs. SVC: superior vena cava; IVC: inferior vena cava; RA: right atrium; RV: right ventricle; rtPA: right pulmonary artery; rtPCW: right pulmonary capillary wedge pressure; LV: left ventricle; Ao: aorta

Fig. 3

Intraoperative gross findings. a The inside of the pericardial sac displays adhesions with some calcification; b A high degree of calcification in the myocardium is observed

Fig. 4

Histopathological findings. a There is a high degree of calcification and fibrosis in the pericardium (green arrow); b Lymphocytic infiltration around the pericardial vessels (blue arrow)