[PAGID] Helicobacter/Flexispira cellulitis in XLA

[Ashish Kumar]

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

Ashish Kumar 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