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March 2002
Presented by: Dr. D. Yamamura1 and Mr. L. Wilcox2

1MDS Laboratories and 2Hamilton Regional Laboratory Medicine Program




A previously healthy 9-year-old female was admitted to Children's Hospital with a 10 day history of fever up to 103ºF and headache. The patient developed a raised red rash with some pustules 7 days prior to admission beginning on her lower extremities and soles of her feet progressing to involve her upper extremities, palms, face and trunk. Multiple joints involving her right hand, left hand, right elbow, left wrist, left shoulder and bilateral knees and ankle joints were red and swollen. The polyarthritis was asymmetric and migratory. The patient denied any history of arthritis. There was no preceding dairrheal or respiratory illness. The patient was not sexually active and had not travelled. There was no significant past medical history. A maternal aunt had rheumatoid arthiritis. The patient was on Acetaminophen and Ibuprofen. All members of her family had a recent upper respiratory infection. The patient had a pet rat.

On physical examination, the patient appeared toxic and listless. She had temperature of 36.9ºC and had an elevated heart rate. The respiratory and cardiovascular examination was unremarkable. Swelling, erythema and decreased ROM were seen in multiple joints. Pustular lesions were seen on the soles of her feet. There was no enlargement of her lymph nodes, or lesions in the oral cavity.

Laboratory investigation revealed that the patient had a WBC 8.3, Platelets 328, ESR 85, CRP 168, and a negative ASOT. Rheumatoid factor was normal. Joint aspirate of her knee showed 45.5 x 109 nucleated cells/L with 89% neutrophils. One of 2 sets of blood cultures using the BACTEC 9240 (Bectom Dickson Microbiology Systems) became positive after 28 hours of incubation. The gram stain revealed a moderate to large, pleomorphic gram negative bacilli (GNB) with long filaments and irregular swellings (Figure 1). Aspirate of the right knee did not reveal any organisms by gram stain and the culture was negative. A pleomorphic GNB was seen on the gram stain of a swab of a pustule on the right foot.

The patient was treated with intravenous penicillin and gentamicin and improved clinically. A transthoracic echocardiogram did not reveal endocarditis. The patient was discharged home on amoxicillin.

Results from further microbiology investigations are summarized. The blood culture was sub-cultured to sheep blood agar (SBA) incubated anaerobically, chocolate agar (CA) incubated in 5% CO2, and MacConkey agar (MAC) with crystal violet incubated aerobically. No growth was seen on MAC or CA. Pinpoint growth was seen at 48 hours on SBA. The colonies were round, smooth, and gray. Initial work-up revealed a catalase and oxidase-negative organism. No reaction was seen on standard biochemical tests. The organism was incubated in supplemented thioglycollate broth (Figure 2). Further biochemical tests were performed. A reference laboratory confirmed the identification.


  1. What are the possible infectious and non-infectious causes of rash, fever, and polyarthritis? After viewing the gram stain what are the most likely cause(s)?

  2. What zoonotic (animal) sources have been linked to this syndrome?

  3. In a routine microbiology laboratory, what further tests and supplementation would aid in the diagnosis of this organism? What reaction do you see with the thioglycollate broth and what organism is most likely given the reaction? What other diagnostic modalities are available to confirm the identification?

  4. What are L-forms and what implication does this have for treatment?

  5. What clinical complications have been reported with this syndrome?


Figure 1: Gram stained smear of isolate on blood agar medium Figure 2: Supplemented Thioglycollate Broth


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