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

[CIS-PIDD]

... In these cases I would look for ADA2 activity (CECR1 mutations).
... just a gut feeling, no evidence...
Yours, Bodo

****************************************
Univ.-Prof. Dr. med. B. Grimbacher

Scientific-Director
CCI-Center for Chronic Immunodeficiency
UNIVERSITÄTSKLINIKUM FREIBURG
Tel.: 0761 270-77731  Fax: -77744
Breisacherstraße 115, 79106 Freiburg
bodo.grimbacher at uniklinik-freiburg.de<mailto:bodo.grimbacher at uniklinik-freiburg.de>
www.uniklinik-freiburg.de/cci


Von: CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Antworten an: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Datum: Monday 21 November 2016 14:56
An: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Betreff: Re: [cis-pidd] Sweet syndrome associated with immune deficiency

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<mailto: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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