[PAGID] Helicobacter/Flexispira cellulitis in XLA. .

[Turvey, Stuart]

Dear Mary-Ellen and PAGID colleagues,

Thank you for your input following my previous post. I am now writing with a brief update.

We admitted the patient last week. His blood culture grew gram negative rods identified as Helicobacter bilis/Flexispira rappini by two different sequencing methods (16S and chaperonin).

We started combination therapy with imipenem, azithromycin and levaquin (as suggested by Mary-Ellen and others) but we have run into a few logistic challenges. Apparently imipenem is too unstable for home iv use and meropenem is a rather expensive alternative (~$80K for 12 months).

The ID team  here at our hospital is wondering if there has been success with alternative antibiotic regimens, particularly because this patient lives on a very remote area island in northern British Columbia.

Thank you once again for your support.

Stuart Turvey


Stuart Turvey MB BS DPhil
Associate Professor
Division of Infectious and Immunological Diseases
University of British Columbia
BC Children's Hospital and Child & Family Research Institute
950 West 28 Avenue
Vancouver BC V5Z 4H4
CANADA
Ph: 604 875 2345 x5094
Fax: 604 875 2226
________________________________________
From: pagid-bounces at list.clinimmsoc.org [pagid-bounces at list.clinimmsoc.org] On Behalf Of Conley, Mary Ellen [maryellen.conley at STJUDE.ORG]
Sent: June-18-10 9:22 AM
To: 'pagid at list.clinimmsoc.org'
Subject: Re: [PAGID] Helicobacter/Flexispira cellulitis in XLA.    .

Hi Stuart,
I would echo Howard Lederman's comments that prolonged therapy, usually IV therapy, is needed for Flexispira.  Patients who have had symptoms for a long time may need longer therapy.  The NIH is now recommending a year (yes a year!!) of IV imipenem 3 times a day plus oral antibiotics (levaquin and azithromycin).  Several patients have had relapses (positive blood cultures or progressive disease) after 6 months of IV therapy.

The question of pyoderma gangrenosa is an interesting one.  In my experience, the swelling and induration starts first and the ulceration comes later in Flexispira infection.  The literature indicates that the ulceration comes first in pyoderma gangrenosa.  I am sure there are exceptions to both.  I think the situation is made more complicated by the fact that the flexispira is very difficult to culture and maybe impossible to culture if the patient is already on oral antibiotics that are partially effective.

I am facinated by this unusual infection in XLA.  To my knowledge, it has only been seen in post-pubertal males and only in lower extremities.  I don't know why.

Everybody - please remember to sign with your full name and institution and city.
Mary Ellen


Mary Ellen Conley, MD
Department of Immunology/ Mail Stop 351
St. Jude Children's Research Hospital
262 Danny Thomas Place
Memphis, TN 38105-3678
FAX  901-595-3977
TEL  901-595-2576


-----Original Message-----
From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Ashish Kumar
Sent: Friday, June 18, 2010 3:27 AM
To: pagid at list.clinimmsoc.org
Subject: Re: [PAGID] Helicobacter/Flexispira cellulitis in XLA. .

As Jason mentioned in his email, Scott Baker and I had a patient with XLA who had chronic ulcers on both lower legs, one extending from the knee down to the ankle (Jason Raasch presented a poster on this at a satellite symposium http://www.clinimmsoc.org/meetings/2006/pidconsort/raasch_jason.pdf).
The history is that it began as a small pustule that opened up and then spread. We saw him when he was 17 and this had been going on for a couple years. We treated him for 6 months on various antimicrobial regimens including 6 weeks of IV Gentamicin, Ciprofloxacin and others with no benefit. We performed several biopsies, all of which failed to detect any pathogens. After seeing Mary Ellen's presentation at the Boston FOCIS meeting a couple years ago and the case report mentioned by Howard, we even checked for Flexispira using several PCR combinations, but those were negative too. A dermatologist suggested pyoderma gangrenosum so we put him on MMF and steroids and within a few months the ulcers completely healed. He was not very compliant with his steroids and every time he missed a few doses, the ulcers would recur.
Unfortunately, last year he developed severe enterocolitis (etiology
unknown) and then Aspergillus in his lungs and brain which led to his demise.

PG is a diagnosis of exclusion, and since a biopsy in this case actually shows organisms, I would treat it with antibiotics. Besides these handful of cases or leg ulcers in XLA, are there others out there that haven't been discussed or reported?

Ashish

 AshishKumar MD, PhD
Assistant Professor
BMT/Immune Deficiency
Cincinnati Children's Hospital

>>> "Turvey, Stuart" <sturvey at cw.bc.ca> 06/16/10 3:18 PM >>>

Dear Colleagues,
I am writing to explore your collective wisdom in planning treatment for a 15 yo boy with X-linked agammaglobulinemia (XLA) who has chronic lower leg cellulitis likely due to infection with a Helicobacter/Flexispira organism.
Briefly, for the past year has had a worsening skin rash/cellulitis on his lower extremities. The swelling and erythema have fluctuated and at times have had an appearance consistent with erythema nodosum. Over time the skin has become 'woody' and indurated. Careful examination of a recent skin biopsy revealed curvilinear rods, and plans are in place for a repeat biopsy with special cultures and 16S ribosomal RNA sequencing to confirm the microbiological diagnosis.
I would appreciate any advice on an optimal antibiotic treatment plan. A major complication is that the family live on a beautiful, but remote island off the northwest coast of British Columbia, Canada.....so a simple but effective antibiotic schedule would be preferable.
Thank you in advance for your input.
Stuart Turvey

Stuart Turvey MB BS DPhil
Associate Professor
Division of Infectious and Immunological Diseases University of British Columbia BC Children's Hospital and Child & Family Research Institute 950 West 28 Avenue Vancouver BC V5Z 4H4 Canada
Ph: 604 875 2345 x5094
Fax: 604 875 2226



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