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

[CIS-PIDD]

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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