[CIS PIDD] [cis-pidd] Sweet syndrome associated with immune deficiency

[CIS-PIDD]

I know a patient with CVID and Sweet Syndrome.

Yesim


Yesim Yilmaz Demirdag, MD
Columbia University Medical Center
New York, NY
(212) 305 2300



On Sun, Nov 20, 2016 at 3:43 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
wrote:

> We have a 25 year old transgendered male with a history of severe Sweet’s
> syndrome that has flared multiple times despite immunosuppression including
> steroids, infliximab, cyclosporine, hyperbaric oxygen, dapsone and
> colchicine.  He requires frequent hospitalizations for flares and does
> respond to high dose IV steroids.  His Sweet’s syndrome has been attributed
> to his testosterone use as there is no other clear trigger (no malignancy,
> no IBD, no other drugs).  He has now been off testosterone for the past 3
> months and continues to flare.  Given the severity of his Sweet’s syndrome,
> we were asked to evaluate whether there might be possible associated immune
> deficiency.  There are reports of Sweet’s syndrome associated with immune
> deficiency but there does not seem to be any clear association with any
> particular disorder but seems to be associated with chronic inflammation in
> the setting of immune deficiency.  He has been on significant
> immunosuppression so his testing is likely to be abnormal (we know he has
> hypogammaglobulinemia but in the setting of long term high dose steroids).
> He has no history of infection prior to his diagnosis of Sweets about 15
> months ago and no significant family history of infection, immune
> deficiency, or autoimmunity.
>
> Thanks,
>
> Rebecca Saff
>
>
>
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