title: Severe axillary lymphadenitis after BCG vaccination: alert for primary immunodeficiencies.
abstract: The bacilli Calmette-Guérin (BCG) vaccine is administered to all newborns in countries where tuberculosis is endemic. Immunocompromised hosts, namely patients with human immunodeficiency virus infection or primary immunodeficiencies, are especially prone to serious complications from this vaccine. We report three cases of BCG disease in children with primary immunodeficiencies: one with a partial recessive interferon-γ receptor 1 deficiency, who developed BCG dissemination; and two relatives with ZAP70 deficiency, a severe combined immunodeficiency, both of whom presented with regional and distant BCG disease. All had severe axillary lymphadenitis. These clinical cases underline the importance of considering the diagnosis of immunodeficiency in a child with severe axillary lymphadenitis after BCG vaccination and of disseminated BCG disease in an immunodeficient child in the appropriate clinical setting. Moreover, BCG vaccination should be delayed in every newborn with a family history of primary immunodeficiency until the condition has been ruled out.
authors.affiliation: Medical Inpatient Department, Coimbra Pediatric Hospital, Coimbra, Portugal. alexandrafigueirasantos@gmail.com
authors.forename: Alexandra
authors.initials: A
authors.lastname: Santos
authors.forename: Andrea
authors.initials: A
authors.lastname: Dias
authors.forename: Ana
authors.initials: A
authors.lastname: Cordeiro
authors.forename: Carolina
authors.initials: C
authors.lastname: Cordinhã
authors.forename: Sónia
authors.initials: S
authors.lastname: Lemos
authors.forename: Graça
authors.initials: G
authors.lastname: Rocha
authors.forename: Emília
authors.initials: E
authors.lastname: Faria
mesh_terms: D001500:BCG Vaccine / Q000008:administration & dosage / Q000009:adverse effects*; D005260:Female; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications*; D007223:Infant; D008199:Lymphadenitis / Q000382:microbiology*; D008297:Male; D009163:Mycobacterium bovis / Q000276:immunology; D017471:Receptors, Interferon / Q000172:deficiency*; D016511:Severe Combined Immunodeficiency / Q000150:complications; D014376:Tuberculosis / Q000175:diagnosis / Q000382:microbiology* / Q000517:prevention & control; D014611:Vaccination / Q000009:adverse effects; D051746:ZAP-70 Protein-Tyrosine Kinase / Q000172:deficiency; D000096743:Interferon gamma Receptor
chemical_list: D001500:BCG Vaccine; D017471:Receptors, Interferon; D051746:ZAP-70 Protein-Tyrosine Kinase; D000096743:Interferon gamma Receptor