[CIS PIDD] [cis-pidd] Severe aphthous ulcers in an adolescent with CVID

Fri Jun 6 13:34:11 EDT 2014

Dear Jane - I have a young adult female patient who has had lifelong oral and genital ulcers that I had previously diagnosed with Behcet's whose infant son is now on the transplant list with a novel mutation in NEMO.  I am thinking she may have skewed X-inactivation.  Are there any suggestions of deficient immune responses in your patient?

Sent from my iPhone

On Jun 5, 2014, at 3:23 AM, "Jane Peake" <j.peake at uq.edu.au<mailto:j.peake at uq.edu.au>> wrote:

Hi all
I have a young girl with CVID who has been plagued with severe disabling aphthous ulcers for many years. Since starting on IVIg her infectious problems have largely resolved and her ulcers are better but they are still problematic. She gets them on her tongue, buccal mucosa and roof of the mouth and when she has them she finds eating and speech very difficult. Episodes are intermittent but she rarely goes more than a couple of weeks without a severe episode. She has had a couple of episodes of labial lesions but since starting doxycycline (on the advice of the gynaecologists) she has only had one episode when they had run out of the doxycycline. We have tried a variety of topical treatments – local anaesthetics and steroids. She responds well to oral steroids but these cannot be used regularly. Does anybody have any other ideas in this setting?
Thanks for any suggestions
Cheers Jane

Dr Jane Peake
Paediatric Immunologist and Allergist
Senior Lecturer
University of Queensland
level 3 Foundation Building
Royal Children's Hospital
Herston Rd, Herston QLD 4029

---

The CIS-PIDD listserv is supported by:

[http://www.clinimmsoc.org/UserFiles/image/cis-pidd-list-logo_v1.jpg]
The science & practice of human immunology

P: +1.414.224.8095
E: info at clinimmsoc.org<mailto:info at clinimmsoc.org>

Not a member of CIS? Please visit www.clinimmsoc.org<https://cis.execinc.com/edibo/Signup> to join!

You are currently subscribed to cis-pidd as: patkinson at peds.uab.edu<mailto:patkinson at peds.uab.edu>.
To unsubscribe click here: http://lm.clinimmsoc.org/u?id=188272311.ffbc45cef69df951d7852b70c6440f49&n=T&l=cis-pidd&o=44974894

---
The CIS-PIDD listserv is supported by the Clinical Immunology Society
The science & practice of human immunology

P: +1.414.224.8095
E: info at clinimmsoc.org

Not a member of CIS? Please visit www.clinimmsoc.org to join!

You are currently subscribed to cis-pidd as: pagid at list.clinimmsoc.org.
To unsubscribe click here: http://lm.clinimmsoc.org/u?id=183939985.3ea13d40a15475ac00ebbd9cd8a37d6d&n=T&l=cis-pidd&o=44980763
or send a blank email to leave-44980763-183939985.3ea13d40a15475ac00ebbd9cd8a37d6d at lists.clinimmsoc.org
-------------- next part --------------
An HTML attachment was scrubbed...
URL: <http://seven.pairlist.net/pipermail/pagid/attachments/20140606/23a0abb8/attachment.html>