A case of Behçet's disease with esophageal ulcers complicated with systemic sclerosis, chronic hepatitis C, and pancytopenia

Abstract

A 62-year-old man was admitted to our hospital because of retrosternal burning pain and high fever in May, 2002. In 1995 chronic hepatitis C was diagnosed. Five years before admission he had been suffering from recurrent oral aphthous ulcers and genital ulcers. Distal scleroderma developed and the diagnosis of systemic sclerosis was made by skin biopsy in 1999. Prednisolone therapy, 5-30 mg/day, had been administered since then. In May 2000, he was referred to our department, and diagnosis of incomplete-type Behçet's disease was made because he had erythema nodosa, oral aphthous ulcers and genital ulcers. Asymptomatic mild pancytopenia was also found. In November 2000, gastrofiberscopy revealed that he had esophageal and gastric ulcers resistant to regular treatment and was diagnosed as entero-Behçet's disease, a subtype of the disease. The activity of esophageal and gastric ulcers was resistant to the low dose glucocorticoid and more than a moderate dose (30 mg/day) of prednisolone was necessary to reduce the activity. His gastrointestinal symptoms fluctuated with low dose prednisolone. Gastrofiberscopy on admission revealed that he had four shallow active oval ulcers in the middle-lower esophagus and distinct blind-fistula in the lower esophagus. Prednisolone were increased to 30 mg/day for his active entero-Behçet's disease, however, his burning retrosternal pain remained. He died on the 81st hospital day due to severe pneumonia. This is a rare case of Behçet's disease complicated with esophageal ulcers, systemic sclerosis, chronic hepatitis C, and pancytopenia. Of interest is the mechanism of coincidence of these diseases from the pathological point of view.