Shewanella species are an unusual cause of disease in humans. However, reports of Shewanella infections have been increasing, and hepatobiliary disease has been proposed as a predisposing factor following a critical course. We report the first Japanese septic case of decompensated liver disease in which this bacterium acted as a definite pathogen. A 67-year-old Japanese man with primary sclerosing cholangitis was admitted to our hospital complaining of fever, general fatigue, pain, and a rash on the lower left extremity. He was tentatively diagnosed with necrotizing fasciitis caused by Vibrio vulnificus because of his decompensated cirrhotic liver and history of consuming raw fish. Thereafter, the diagnosis was altered to cellulitis and Shewanella septicemia on the basis of the characteristics of his skin lesion and an arterial blood culture. He died of multiple organ failure on the eleventh day in the hospital. Since several reports have demonstrated that Shewanella can cause lethal sepsis in patients with hepatobiliary disease, we should be aware of the pathogenicity of this bacterium.

We experienced a case of blue rubber bleb nevus syndrome with familial onset. The patient was a 32-year-old male with a gallstone and many bluish rubber bleb-like hemangiomas on the skin. He suffered from repeated rectal bleeding and underwent a sigmoidectomy at age 17. Gastrointestinal hemangiomas were recognized in the esophagus, stomach, ileum and colon. An angiogram revealed multiple small poolings in the liver, suggesting the presence of hemangiomas. During the cholecystectomy, surgeons noted the presence of hemangiomas on the surface of the liver, serosa of the small intestine and retroperitoneum. Out of 73 blood relatives, 24 also had bluish skin hemangiomas, suggesting them to be inherited by an autosomal dominant trait. More than sixty cases of this syndrome had been reported in the world, eight of which had family histories of skin lesions. However, in only three cases, including our own, was the presence of skin and gastrointestinal hemangiomas recognized. Because the clinical indications for diagnosis of blue rubber bleb nevus syndrome consist of minimal to massive bleeding from the gastrointestinal tract, the possibility that this syndrome is present should be considered when diagnosing a bleeding patient with multiple bluish rubber bleb-like skin lesions, in addition to taking a detailed family history.

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