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

[CIS-PIDD]

Do your patients smoke?
To my knowledge Hidradenitis suppurativa is exacerbated by smoking...
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
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: Tuesday 16 August 2016 19:32
An: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
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<mailto: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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