Multiple recurrent abscesses in a patient with undiagnosed IL-12 deficiency and infection by Burkholderia gladioli

Abstract

We report the occurrence of two severe illnesses experienced by one patient over a 19 year period of time. Both illnesses were characterized by severe inflammation and tissue destruction. Signs and symptoms of the first illness were characteristic of lymphogranuloma venereum (LGV). The second illness mimicked scrofula. During the second illness the patient was discovered to have a rare immunodeficiency due to auto-antibodies to Interleukin (IL)-12 and infection by Burkholderia gladioli, a plant pathogen usually harmless in humans.We were able to retrieve biopsies from the first illness to establish that B. gladioli was already present during the original presentation. That first illness lasted 5 year s, but she survived without the correct pathogen ever being identified, and without a diagnosis of immunodeficiency. After a remission of 10 year s, she experienced her second illness.The responses to treatment before and after the correct diagnoses were established provide us with an excellent opportunity to consider and discuss how disease expression reflects complex relationships between host defenses and microbial characteristics. Keywords: Immunodeficiency syndrome, Burkholderia gladioli, Interleukin (IL)-12, Lymphogranuloma venereum (LGV), scrofula