A case of primary biliary cirrhosis complicated by Behçet's disease and palmoplantar pustulosis

Abstract

A 46-year-old woman was diagnosed with palmoplantar pustulosis (PPP) at the Department of Dermatology, Fukushima Medical University Hospital in 2000, and was treated with ointment. However, because liver dysfunction developed in 2003, she was referred to our department, where primary biliary cirrhosis (PBC) was also diagnosed on the basis of clinical findings. One year later, at the age of 49, she developed manifestations of Behçet's disease (BD), including erythema nodosum in the lower extremities. Because she had a history of uveitis, recurrent oral ulceration was present, and the HLA typing was positive for B51, BD was additionally diagnosed. Liver function normalized within three months of the start of treatment with ursodesoxycholic acid (UDCA). This is the first case of PBC associated with BD and PPP.

Figure 1

Computed tomographic angiography shows aneurysms of the left. internal carotid-posterior communicating artery, left. anterior communicating artery, and right. posterior cerebral artery (arrows).

Figure 2

Typical eruption of erythema nodosum shows erythematous nodules on the anterior aspect of the leg.

Figure 3

Liver biopsy specimen shows slightly enlarged portal tracts with no evidence of chronic nonsuppurative destructive cholangitis. Hematoxylin and eosin, original magnification ×100.