ABSTRACT
Primary biliary cirrhosis is a rare progressive autoimmune liver disease that causes chronic cholestasis. Of patients with primary biliary cirrhosis, 75% develop secondary Sjogren syndrome and could develop vitamin A deficiency. Here, we report the case of a patient with primary biliary cirrhosis who developed a secondary Sjogren syndrome and vitamin A deficiency, which led to severe and unusual eye involvement with multiple and recurrent spontaneous corneal perforations. Corneal perforations in patients with primary biliary cirrhosis and secondary Sjogren syndrome are rare but devastating complications, in contrast to other eye clinical manifestations of the disease.
Keywords:
Primary biliary cirrhosis; Corneal perforation; Sjogren syndrome; Vitamin A deficiency; Autoimmune disease