We present the case of a male infant diagnosed with IPEX (immune
dysregulation, polyendocrinopathy, enteropathy, and X-linked) syndrome
who presented a generalized rash with a BCG site reaction several months
after vaccination. In our patient this
dermatological manifestation might be secondary to immune dysregulation,
given the important role of Tregs and Th17 cells in mycobacterial
immunity, similar to that observed in Kawasaki disease patients.