Disseminated Bacille Calmette-Guerin (BCG) infection following vaccina
tion with live BCG may occur in children without any well-defined immu
ne deficiency. Children with unexplained disseminated infection due to
environmental non-tuberculous mycobacteria (NTM) have also been repor
ted. Several lines of evidence suggested that such idiopathic BCG and
NTM infections may represent different complications of the same autos
omal recessive inherited immune disorder. Accordingly, gamma-interfero
n receptor deficiency was recently identified in three kindreds with N
TM and BCG disseminated infection. This article reports the current kn
owledge on idiopathic BCG or NTM disseminated infections, and the unde
rlying inherited immune deficiencies.