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

[CIS-PIDD]

She and her five siblings, all with similar problems, and her parents have
had WGS at NIH and they are still analyzing the data.
Richard

On Wed, Jun 21, 2017 at 11:26 AM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
wrote:

> Is she genetically negative for all autoinflammatory genes?
>
> Anti il1 blockers seems as a good option.
>
> Leo
>
> Sent from my iPhone
>
> On 21 Jun 2017, at 12:19, 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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-- 
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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