[CIS PIDD] [cis-pidd] AR-CGD and Hidradenitis

Tue Aug 16 17:11:15 EDT 2016

Thanks everyone!  I will look into the idea of intralesional steroids. 
I appreciate the caution on other immunomodulatory drug like Humira.  

The transplant question did come up.  It would certainly fix the CGD
issue.  It would be interesting to see if it would help the hidradenitis
issue directly or indirectly.  My patient(s) don't have many
well-matched donors.  They have many 8/10 matched unrelated donors and
perhaps only one 9/10 matched unrelated donor.  A haplo approach
(parents are carriers) with post-transplant cytoxan would be possible. 
I would love to chat offline if anybody has thoughts on how best to
proceed with that.

Dave

>>> "CIS-PIDD" <cis-pidd at lists.clinimmsoc.org> 8/16/2016 1:47 PM >>>
Just to be provoking, and to follow Jennifer’s idea, I would say that
the best treatment for CGD is Haematopoietic Stem Cell Transplantation…

Elie Haddad, MD, PhD,

Montreal, Canada
Ph: 1 514 345 4713
fax: 1 514 345 4897
e-mail: elie.haddad at umontreal.ca

Le 2016-08-16 à 16:39, CIS-PIDD <cis-pidd at lists.clinimmsoc.org> a écrit
:

I would avoid using anti anti-TNF agents in CGD patients

Jen

________________________________________
From: CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
Sent: Tuesday, August 16, 2016 2:28 PM
To: CIS-PIDD
Subject: RE: [cis-pidd] AR-CGD and Hidradenitis

In the US, adalimumab (Humira) is approved for hidradenitis
suppurativa...

Tony Infante

-----Original Message-----
From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Sent: Tuesday, August 16, 2016 1:05 PM
To: CIS-PIDD
Subject: [BULK] AW: [cis-pidd] AR-CGD and Hidradenitis

Dear Joe and Dave,

I never had a case like this, but had very good results in CGD related
inflamamtion/infection (panniculitis, liver abscess, thymic mass) with
low dose prednisolone (1-2 mg/kg).

Best regards, Fabian

Fabian Hauck, MD, PhD

Attending physician / Head Immunodeficiency Unit and Immunological
Diagnostics Laboratoy Pediatrics / Pediatric Hematology and Oncology /
Immunology (DGfI)

Dr. von Hauner Children's Hospital
Klinikum der Universität München
Lindwurmstr. 4, 80337 München
Germany

Tel.: +49 89 4400-53931
Fax: +49 89 4400-53964
E-Mail: fabian.hauck at med.uni-muenchen.de
________________________________________
Von: CIS-PIDD [cis-pidd at lists.clinimmsoc.org]
Gesendet: Dienstag, 16. August 2016 19:32
An: CIS-PIDD
Betreff: Re: [cis-pidd] AR-CGD and Hidradenitis

Hi Joe,

I am very interested in your case and the responses from colleagues.  I
have basically a duplicate case.  I am not sure what to do with her.

She is a 21 year old who came to use with Hidradenitis Supurativa s/p
resection (non-healing wound) in her right axilla.  She was set up to
donate marrow for her little brother who had AR CGD.  We did a DHR on
her...  and her DHR was also abnormal.  Mutation analysis documented
homozygous NCF1 mutations.  She was placed on itraconazole, bactrim and
actimmune.  She is compliant.  I treated her with a prolonged course of
doxycycline - which helped her left axilla (the one that was not
operated on) as she some draining wounds on that side.  The problem is
the right side that had the surgery.  It is a clean wound that is
approxiamtely 1.5 inches in length and a mm or so deep.

Her inflammatory markers have remained mildy elevated ESR 60-70s.  CRP
also elevated.  We scanned her chest looking of other things - negative.
 I did a MR of her right axilla - no evidence of a deeper infection.  I
tried a course of corticosteroids for a few months which helped a tiny
bit, but she got pretty toxic.  I put in a PICC line and placed her on
ertapenem for about 1.5 months since a swab from the lesion grew
serratia and staph.  The wound did not improve on antibiotics and nor
did her ESR and CRP.

Our plastics team offered her a "flap" procedure assuming this is just
hidradenitis.  I was not that excited about that as we may run into more
wound healing issues.  I wanted to make sure we have exhausted other
options.

-Dave

"CIS-PIDD" <cis-pidd at lists.clinimmsoc.org> 8/16/2016 10:19 AM >>>
Colleagues:

I am consulting on a 19yo F with autosomal recessive-CGD (NCF-1
mutation).  She has been remarkably well.  She presented at ~10yo with
cervical adenitis and severe gingivitis.  Since that time she has been
treated with TMP-SMX, itraconazole and interferon-gamma.  She has had no
other episodes of adenitis or any other infection.  1 year ago she
developed bilateral axillary hidradenitis suppurativa.   She has had
excisional surgery on the left side.  Currently, both areas are scarred
with minimal discharge.

Any suggestions?

Joe Church, MD
Children's Hospital Los Angeles

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