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

Tue Aug 16 14:51:42 EDT 2016

I would consider intra lesion steroids.  I have a couple of CGD patient with inflammatory cystic acne that would not heal and this therapy has helped.  It might be an option to try prior to systemic therapy.
Just a thought

Jason W Caldwell DO FAAAAI
Associate Professor of Internal Medicine and Pediatrics
Section of Pulmonary, Critical Care, Allergic and Immunological Diseases
Director of Allergy/Immunology
Program Director of Allergy/Immunology Fellowship
Wake Forest University School of Medicine
Office: 336-716-5166
Administrative: 336-716-4843
Pager: 336-806-8330
jcaldwel at wakehealth.edu

-----Original Message-----
From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org] 
Sent: Tuesday, August 16, 2016 2:05 PM
To: CIS-PIDD
Subject: 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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