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

Fri Aug 19 11:35:58 EDT 2016

I have been following this thread with interest without having any expertise in treating hidradenitis suppurativa. I am wondering whether any of you with an expertise in this condition could answer a simple question that may pave the way to treating this patient and others who have a complicated course with hidradenitis suppurativa.
Since hidradenitis suppurativa is a locally limited condition, as opposed to psoriasis, RA or IBD that require systemic treatment, could the blockade of the incriminated pathway in the lesional tissue be considered? Has any attempt been made to treat this condition with intralesional deposition of any of the TNF-alpha antagonists or ustekinumab or secukinumab for IL-23 and Th17 to minimize the systemic effects while providing sufficient benefit at the site of disease to allow healing?
Best,Soheil
SoheilChegini, M.D.

ExtonAllergy & Asthma Associates

656West Lincoln Hwy.

Exton,PA 19341

Phone:(610) 269-3066

Fax:(610) 269-8615

      From: CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
 To: CIS-PIDD <cis-pidd at lyris.dundee.net> 
 Sent: Friday, August 19, 2016 5:54 AM
 Subject: Re: [cis-pidd] AR-CGD and Hidradenitis

 Would love to hear of any real life experiences from list members??

Expression of the IL-23/Th17 pathway in lesions of hidradenitis suppurativa.
Schlapbach C, et al. J Am Acad Dermatol. 2011.
Authors
Schlapbach C1, Hänni T, Yawalkar N, Hunger RE.
Author information

   -    1Department of Dermatology, Inselspital, University of Bern, Bern, Switzerland.

Citation
J Am Acad Dermatol. 2011 Oct;65(4):790-8. doi: 10.1016/j.jaad.2010.07.010.
Mikko
Oyl Mikko SeppänenHarvinaissairauksien yksikkö (HAKE)
Head, Rare Disease Center,Helsinki University Hospital (HUH)FINLAND
phone +358 947180201GSM +358 50 4279606fax +358 9 47174703
CIS-PIDD <cis-pidd at lists.clinimmsoc.org> kirjoitti 19.8.2016 kello 12.22:

Hi,
What do you think of ustekinumab?
There is some experiences in the literature for HS (http://www.ncbi.nlm.nih.gov/pubmed/21605174) and it is generally considered (at least in psoriasis) safer then anti-TNF.
Interestingly a case of CGD-colitis succesfully treated with ustekinumab was recently reported (http://link.springer.com/article/10.1007%2Fs10875-016-0318-x ).
It also (kind of) reminds me the case presented by Steven Hollande in his lecture at CIS Boston 2016 of the use of ustekinumab in LAD ( https://cis.confex.com/cis/2016/videogateway.cgi/id/593?recordingid=593 ).
Kind regards,
Boaz Palterer, MDResident in Allergology and Clinical ImmunologyUniversity of Florence, Italyemail boaz.palterer at gmail.com

On Thu, Aug 18, 2016 at 3:43 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org> wrote:

Thanks, Mikko.  There is no family history of HS.  I very much appreciate the references.  JC From: CIS-PIDD [mailto:cis-pidd at lists. clinimmsoc.org]
Sent: Wednesday, August 17, 2016 9:52 PM
To: CIS-PIDD
Subject: Re: [cis-pidd] AR-CGD and Hidradenitis Joe, Tricky to treat then!! More of a theoretic thought than anything practical: of course her HS disease may "solely" be caused by CGD, but being lean and non-smoking, does she have any family history of HS?  Some monogenic HS cases are caused by LOF gamma-secretase complex gene mutations possibly affecting Notch-signaling (Dermatol Clin. 2016 Jan;34(1):23-8. doi: 10.1016/j.det.2015.07.002). There are other suspected loci, CNVs and unknown genes as well. g-secretase mut carriers tend to have early, prepubertal onset. Lesionally and perilesionally, there are increased Th17 and gamma-interferon secreting CD4 cells a.o.t. Follicles secrete increased amounts of IL-1b a.o.t. (
PMID
 27206704), so maybe there will be more trials of biologicals and topical targeted immunomodulation in the future? Not that this helps now... Mikko Oyl Mikko Seppänen Harvinaissairauksien yksikkö (HAKE) Head, Rare Disease Center,Helsinki University Hospital (HUH)FINLAND phone +358 947180201GSM +358 50 4279606fax +358 9 47174703
CIS-PIDD <cis-pidd at lists.clinimmsoc.org > kirjoitti 18.8.2016 kello 2.35:
Colleagues:

Thank you all for the great suggestions.  

Thalidomide is a good idea.  The problem is that this young lady refused Accutane (isotretinoin) because she would have to take birth control pills and have regular lab monitoring done.  However, I will mention this to the primary MD.

As noted, anti-TNF treatments have been associated with serious complications in CGD patients.

I discussed with her the potential for transplant to cure her disease.  She is not interested.  She notes that she has not had a single serious infection since her diagnosis and starting prophylaxis, and is unwilling to run the risk.  She may have a mutation that allows some oxidase expression.  I am waiting for results of our in-house DHR.

Injectable or low-dose systemic steroids would address the hyperinflammatory state in CGD.  Either may be the safest place to start.

She does not smoke; she is Asian-American; and lean.  So, except for CGD she has none of the predisposing factors for hidradenitis..

Again, thank you for your assistance.

Joe Church
Children's Hospital Los Angeles

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