[CIS PIDD] [cis-pidd] pyoderma gangrinosum and pathergy

[CIS-PIDD]

Richard, 
I recently had a good experience with anakinra in an infant who had severe PG that is so far idiopathic. Total resolution. 

 I am not sure whether it has latex and any component, but it does come in a glass syringe. 

It has the other advantage that you may not have to give it as a subcut which could cause increased pathergy and more lesions. But rather could be given as IV, since she has an indwelling line. Also easy to combine with other immunosuppressants such as MMF. 
Mona 
___________________________
Mona Riskalla, MD, MS 
Assistant professor of pediatrics
Pediatric Rheumatology
University of Minnesota

> On Jun 21, 2017, at 10:19 AM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org> wrote:
> 
> Colleagues:
> 
> I write for help with a 14 yo girl who doesn't have PID. She has multiple atopic diatheses (asthma, allergic rhinitis, IgE mediated food allergy, latex allergy, FPIES and eczema). In addition to routine treatments for these problems, she receives Xolair and has required calcineurin inhibitors to manage eczema. She experienced DRESS syndrome and hypertension attributed to cyclosporin A. Her eczema has been controlled with mycophenolate. Mycophenolate has been stopped in preparation for treatment with a TNF inhibitor (see below) causing the eczema to flare. She also has hypoadrenalism. Her cumulative lifetime dose of glucocorticoids is high. 
> 
> Because she is unable to feed enterally (she gets severe vomiting and diarrhea with all elemental diets), she is TPN dependent. Maintaining access for TPN has become problematic because of pyoderma gangrinosum and pathergy around the catheter insertion sites. The immunodermatologist had planned to use a TNF inhibitor but the drugs that have been used in children are all packaged with latex stoppers or syringes.
> 
> The questions are:
> 1. Does anyone have experience with biologics useful for pyoderma gangrinosum and pathergy that are not packaged with latex?
> 2. Will it be possible to use a biologic for pyoderma gangrinosum and pathergy that will not flare the eczema?
> 
> Thank you in advance for your input.
> Richard Wasserman
> Dallas
> 
> -- 
> Richard L. Wasserman, MD, PhD
> Allergy Partners of North Texas
> 7777 Forest Lane, Suite B-332
> Dallas, Texas 75230
> Office (972) 566-7788
> Fax (972) 566-8837
> Cell (214) 697-7211
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