[PAGID] Helicobacter/Flexispira cellulitis in XLA

[raas0027 at umn.edu]

Stuart, one thing to consider (now or down the road depending on response 
to antibiotics) is pyoderma gangrenosum (PG).

There are a number of case reports of PG in patients with XLA and I have 
talked to a few PAGID members who have experience with this.

Ashish Kumar had such a patiet a several years ago. Perhaps similar lesion 
to what you are describing. Biopsy cultures grew Psuedomona, staph and 
aceinetobacter. Antibiotics for these organisms and empiric antibiotics for 
atypical mycobacteria were ineffective. Once PG was entertained, the 
patient had dramatic improvement on steroids and mycophenolate.

Maybe not the first thing in your differential but might be important to 
have on the list.

Keep us posted!

-Jason


On Jun 16 2010, Turvey, Stuart wrote:


>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

>


-- 
Jason Raasch, MD

Midwest Immunology Clinic
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