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

[CIS-PIDD]

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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