Clinical presentation

A 37-year-old man complained of a 7 months’ history of yellowish skin and sclera accompanied by fatigue, pruritus and dry mouth, with a history of subcutaneous masses on his elbow. Hard and fixed subcutaneous masses were noted on his palm and periauricular region, the largest of which was approximately 2 cm in diameter (figure 1A). Mildly tortuous veins on his abdominal wall above the umbilicus were detected (figure 1B). The patient had no history of liver disease. Family history was not contributory. Laboratory tests demonstrated a leucocyte count of 2.9×109/L (3.97–9.15×109/L), 7.9% eosinophil (0.5%–5%), a total bilirubin of 94.5μmol/L, gamma-glutamyl transpeptidase of 67.8 U/L (11–50 U/L) and serum IgG of 23.3 g/L (7–16 g/L), IgG4 of 3.48 g/L (0.08–1.4 g/L) and IgE of 256 IU/mL (0–100 IU/mL). Coagulation function tests revealed prothrombin time of 14.8 s (9.4–12.5 s), international normalised ratio (INR) of 1.33 and …