J Rheum Dis.  2011 Mar;18(1):50-54. 10.4078/jrd.2011.18.1.50.

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

• Hwang JY
• Yoon H
• Kang JS
• Lee SA
• Lee WJ
• Lee KU
• Park JY

• KMID: 2223183
• DOI: http://doi.org/10.4078/jrd.2011.18.1.50

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.

Keyword

Primary biliary cirrhosis; Undifferentiated connective tissue disease; Raynaud's phenomenon; Necrosis; Fingers; Diabetes mellitus

MeSH Terms

Aged
Arthritis, Rheumatoid
Autoimmune Diseases
Connective Tissue Diseases
Diabetes Mellitus
Diabetes Mellitus, Type 2
Female
Fingers
Humans
Liver Cirrhosis, Biliary
Lower Extremity
Necrosis
Raynaud Disease
Scleroderma, Systemic
Upper Extremity

Figure

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  Figure 1. Computed tomography of the upper abdomen (A) showed cirrhosis of the liver and spleno-megaly. An angiogram of the left hand (B) revealed slightly decreased blood flow in the palmar branch of the left ulnar artery with smooth tapering, which suggests a normal variant of palmar circulation. (C) The patient's left 5 th finger tip was swollen and showed necrotic changes.

Reference

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