Degos disease

Synonyms:

Malignant atrophic papulosis

Diagnosis:

Clinical:

  • Skin, brain & bowel vasculitis
  • Skin: initially red papules then, umbilicated papules, +white centre & telangiectatic rim
  • Bowel: GI bleed, bowel perforation

MRI:

  • Infarcts
  • T2 hyperintensities, +small hemorrhagic areas
  • T1 +GAD: dural enhancement may occur

Cather Angiogram/angiography:

  • Vasculitis features
  • Peripheral aneurysms
  • Also stenosis in GI arteries e.g. coeliac artery