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BCGitis in IPEX syndrome
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  • Lina Maria Castano-Jaramillo,
  • Carla Toledo-Salinas,
  • Marco Yamazaki-Nakashimada,
  • Marimar Saez-de-Ocariz,
  • Sonia Toussaint-Caire,
  • Juan Carlos Bustamante-Ogando,
  • Francisco Eduardo Rivas-Larrauri,
  • Agustin Mendez-Herrera,
  • Selma C Scheffler-Mendoza
Lina Maria Castano-Jaramillo
Instituto Nacional de Pediatria
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Carla Toledo-Salinas
Instituto Nacional de Pediatria
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Marco Yamazaki-Nakashimada
Instituto Nacional de Pediatria
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Marimar Saez-de-Ocariz
Instituto Nacional de Pediatria
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Sonia Toussaint-Caire
Hospital General Doctor Manuel Gea González
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Juan Carlos Bustamante-Ogando
Instituto Nacional de Pediatria
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Francisco Eduardo Rivas-Larrauri
Instituto Nacional de Pediatria
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Agustin Mendez-Herrera
Instituto Nacional de Pediatria
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Selma C Scheffler-Mendoza
Instituto Nacional de Pediatria
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Abstract

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. Erythema and induration at BCG site might be due to BCG which is a known complication of inborn errors of immunity with susceptibility to mycobacterial infection. However, 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.