Description

A 65-year-old woman presented with severe pain in the fingers of both hands (figure 1).

• Download figure
• Open in new tab
• Download powerpoint

Figure 1

Hands of the patient with cryoglobulin-induced gangrene.

The patient was diagnosed with hepatitis C virus (HCV), following blood transfusion 30 years ago, and had developed the associated pathologies of cirrhosis and cryoglobulinaemia. Over the past 7 years, she had required multiple hospital admissions to treat associated complications. These consisted of renal impairment, hypertension, restrictive cardiomyopathy, ascites, severe peripheral oedema, neuropathy and skin ulceration. Her digital ischaemia was treated with opiate analgesia, calcium channel blockers and repeated prostacyclin infusions with limited benefit aside from pain relief.

HCV is primarily associated with type II mixed cryoglobulinaemia. It is reported that 40–60% of patients with HCV infection have cryoglobulins.1 However, in only 5–10% of cases will a cryoglobulinaemia induced vasculitis and/or intravascular immune complex depositions occur. The prevalence is increased in patients with long standing HCV infection, cirrhosis and in females. The incidence rate for haematological cryoglobulinaemia is quoted to be 2.3–3.0% per year, in which around 0.3% per year develop a vasculitic syndrome, like our patient.2 An important feature is that in many cases the extrahepatic manifestations of HCV infection can occur in patients without features of an overt liver disease.1

HCV infection, particularly when chronic and in the presence of cryoglobulinaemia, is frequently a devastating multisystem disease.3 Around 10% of patients with chronic HCV infection may also go on to develop a malignant B cell lymphoma.

Our patient passed away 24 h after this photograph was taken.