[CIS-PAGID] FW: XLA / helicobacter

[Dowling, Paul MD]

Dear Charlotte,

I also had a case like this. This patient had a diagnosis of XLA and was doing well until age 16 when he started having unexplained painful "bumps" on his arms and legs. They were small at first, warm, painful and would last a few days on his arms and lower legs.  No fever, change in CBC etc. was seen. they initially would clear completely after a few days. He thought they occured from trauma as he played soccer and they occurred in areas usually hit by the ball. Later as they became more frequent they were thought to be some type of vasculitis or atypical cellulitis. Attempts at culture and biopsy showed no organisms. He was treated with a large variety of antibiotics by ID without improvement. Bx was consistent with panniculitis.  A couple lesions on the lower legs persisted and started necrosing...leaving large non healing ulcers. After speaking with a couple experts on XLA the thought of Heliobacter/Flexispira was entertained although all attempts at culturing were futile.   The pateint was eventually sent to the NIH where cutures were also negative but the flexispira organisms were seen by staining the biospies with "Warthin-Starry ?" Stain. He was treated with a PICC line with IV Tobramycin and Meropenem for many months. His lesions started healing. During treatment Tobra was dc'ed as he developed tinnitus and documented hearing loss and was switched to po doxycycline along with the IV Meropenem. He was treated about 10 months until he stopped meds himself and remained well for approximately a year until lost to followup.

Hope this adds a little to your data base on these patients.

Paul

Paul J Dowling, MD
Training Program Director, Allergy/Immunology
Associate Professor, Dept.of Pediatrics
University of Missouri - Kansas City
Children's Mercy Hospitals and Clinics
816-234-3097 (office); email pdowling at cmh.edu


________________________________
From: Ciaccio, Christina, E
Sent: Saturday, July 23, 2011 5:09 PM
To: Dowling, Paul MD
Subject: Fwd: [CIS-PAGID] XLA / helicobacter


Did you have a case like this?

Begin forwarded message:

From: "Charlotte Cunningham-Rundles at mssm.edu<mailto:Cunningham-Rundles at mssm.edu>" <charlotte.cunningham-rundles at mssm.edu<mailto:charlotte.cunningham-rundles at mssm.edu>>
Date: July 23, 2011 9:38:23 AM CDT
To: PAGID <pagid at list.clinimmsoc.org<mailto:pagid at list.clinimmsoc.org>>
Subject: [CIS-PAGID] XLA /  helicobacter
Reply-To: "pagid at list.clinimmsoc.org<mailto:pagid at list.clinimmsoc.org>" <pagid at list.clinimmsoc.org<mailto:pagid at list.clinimmsoc.org>>

Hi,

We have a case of a 25 year old man with leg swelling, pyoderma gangrenosum,  gram neg bacteremia but no fever or increased WBC,   that I think it will prove to be a Helicobacter. He is not yet proven to have XLA but has no B cells and I assume with profound hypogammaglobulinemia and early onset, this is likely what he has.

  I know there are cases reported around the US and world, but wonder, if we as a group:


 1.  Can make a rough count of the cases?
 2.  Say what antibiotics worked in the long run?
 3.  Are there any cases in CVID or other congenital immune defects?

Charlotte

Charlotte Cunningham-Rundles MD PhD
Department of Medicine
Immunology Institute
Mount Sinai School of Medicine
1425 Madison Avenue
New York City, New York 10029

212 987 5593 fax
212 659 9268 telephone


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