[CIS PIDD] [cis-pidd] AW: CGD carriers with mucositis

[CIS-PIDD]

Dear James,

we have done allogeneic bone marrow transplantation in a X-CGD female carrier with non-random X-chromosome inactivation and therapy-refractory inflammatory bowel disease. You could check the degree of X-chromosome inactivation by DHR-123 testing  first and reevaluate histopathology (pigment-laden macrophages etc. ) and then see if transplantation might be an option (PMID: 26464403).

Best regards, Fabian

-----Ursprüngliche Nachricht-----
Von: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org] 
Gesendet: Mittwoch, 24. August 2016 22:32
An: CIS-PIDD
Betreff: [cis-pidd] CGD carriers with mucositis

I replied to a post about scid so resending

I have a quick question about CGD carriers

I have a mother of a son with X-linked CGD who has had horrible oral mucositis as well as discoid lupus on her arms.  I know this can occur in carriers.  She dealt with this her entire life.  No other infections.

She has been tried on plaquenil, Imuran, topical steroids, and oral steroids (as a teenager, stopped since she didn't like the side effects)

Anyone care for moms of CGD patients with similar symptoms and if so has anything worked?

We are going to try a short course of steroids, and then perhaps colchicine.  Our next thought was TNF blockers (a little nervous with her SLE like rashes that things could worsen but if nothing else works we may try this.)

Thanks

James


James W. Verbsky M.D./Ph.D.
Associate Professor of Pediatrics and Microbiology Medical Director, Clinical Immunology Research Laboratory Medical Director, Clinical and Translational Research Medical College of Wisconsin Milwaukee, WI

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