The Primary Biliary Cirrhosis Presenting with Raynaud's Disease and Digital Necrosis

ABSTRACT

Necrosis of the upper extremity occurs rarely in type 2 diabetic patients compared to lower extremity necrosis. We report a 69-year-old woman with type 2 diabetes mellitus who presented with necrosis of the left 5th finger tip. The patient had primary biliary cirrhosis accompanied by necrosis of the fingertip due to severe Raynaud's phenomenon. Primary biliary cirrhosis (PBC) is a typical autoimmune disease, which can in rare cases be accompanied by autoimmune symptoms including sicca symptom and Raynaud's phenomenon. Furthermore, autoimmune diseases, such as systemic sclerosis, rheumatoid arthritis and undifferentiated connective tissue disease (UCTD) can be associated. Although every type of vascular etiology should be considered as a cause of digital necrosis, Raynaud's phenomenon is usually not considered in diabetes. We report this case of finger tip necrosis due to severe Raynaud's phenomenon accompanied by PBC and UCTD in a diabetic patient.