[PAGID] sterile granuloma in NBS1?

[dmvascon at usp.br]

Dear Javier

Here in Brazil I've seen cellular PID patients (not Nijmegen's) with  
several types of Mycobacteria (tuberculosis, avium-intracellulare,  
kansasii, chelonei, abscessus and marinum) but also Rhodococcus equi,  
Nocardia and Actinomyces, that are also acid-fast bacilli when stained  
with Ziehl-Nielsen.

Certainly Wegener's and certain lymphomas (midline destructive  
granuloma (or destructive disease), as well as Langerhans cell  
hystiocytosis enter in the differential diagnosis.

All the best,

Dewton Vasconcelos


Citando "Chinen, Javier" <jxchinen at texaschildrens.org>:


> Dear all,

> I take care of an 18 yo male with Nijmegen syndrome, has CD4s at  

> ~200, poor but not absent mitogen responses, IVIG dependent. No  

> other major organ problems. He has been relatively well on IVIG and  

> Bactrim prophylaxis, with infrequent courses of  

> quinolones/macrolides for upper respiratory infections. Over the  

> last 2 years, he has developed a granulomatous process that started  

> on his nasal septum and at the nare opening, then perforated palate,  

> and after a few months presented with granulomas in his right wrist  

> synovia and radius head, and right distal femur. ENT and Ortho  

> removed diseased tissue, but this progressed and was recurrent in  

> the wrist. Our working diagnosis is mycobacterial disease, based on  

> few, rare acid-fast bacilli that never grew in culture. Multiple  

> biopsies have been tested for mycobacterial DNA by PCR and was  

> negative. Because clinical and epidemiological data, we considered  

> this could be leprosy, however the granulomatous processes continued  

> while on Dapsone and Rifampin for over 3 months, now he is on  

> anti-atypical mycobacterial treatment, though poorly tolerated. He  

> does not have fever, or other systemic symptoms. His CRP is modestly  

> elevated, the granuloma biopsies are reported as a mix of caseating  

> and not caseating, and are well organized. There is no monoclonal  

> population in the granulomas or peripheral blood.

> My questions are;

>

>  1.  Has anyone seen Nijmegen Sx developing sterile granulomatous  

> processes, (excluding related to lymphoma)?

>  2.  Any other diagnosis? Would you use steroids?

>

> Thank you,

>

> Javier

>

>

>

>

>

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