Bacteremia and Lower Limb Skin Lesion in a Patient with X-Linked Agammaglobulinemia-A Case Report and Review of the Literature.

We describe the first case of infection with in a patient with X-linked agammaglobulinemia. A 22-year-old male with X-linked agammaglobulinemia presented with fever, malaise and a painful skin lesion on the lower left extremity. Spiral shaped Gram-negative rods were isolated from blood cultures and later identified as . The patient was treated with various intravenous and oral antibiotic regimens over a period of 10 months, each causing seemingly full clinical and paraclinical remission, yet several episodes of relapse occurred after cessation of antibiotic treatment. The review of the literature showed that only a few cases of infections with enterohepatic helicobacters belonging to the taxons have previously been reported. The majority of cases included patients with X-linked agammaglobulinemia and the symptomatology and course of disease were similar to the case described here. Infections with enterohepatic helicobacters, including , should be considered in patients with X-linked agammaglobulinemia presenting with fever, malaise and skin lesions. Careful cultivation and microbiological investigation are essential to determine the diagnosis and a long treatment period of over 6 months must be expected for successful eradication.