Meningoencephalitis caused by varicella-zoster virus reactivation in a child with dominant partial interferon-gamma receptor-1 deficiency.

We describe a previously symptom-free 13-year-old boy with dominant partial interferon-γ receptor-1 deficiency and unusual varicella-zoster virus reactivation. This case supports the unsettled notion that some interferon-γ R-deficient patients are at enhanced risk for viral disease.

Clinical features of dominant and recessive interferon gamma receptor 1 deficiencies.

Background: Interferon gamma receptor 1 (IFNgammaR1) deficiency is a primary immunodeficiency with allelic dominant and recessive mutations characterised clinically by severe infections with mycobacteria. We aimed to compare the clinical features of recessive and dominant IFNgammaR1 deficiencies.

Methods: We obtained data from a large cohort of patients worldwide.