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Volume 43, Number 6, December 2010

Severe Axillary Lymphadenitis After BCG Vaccination: Alert for Primary Immunodeficiencies

Alexandra Santos, Andrea Dias, Ana Cordeiro, Carolina Cordinhã, Sónia Lemos, Graça Rocha, Emília Faria

Received: December 18, 2008    Revised: February 27, 2009    Accepted: April 17, 2009   


Corresponding author:

Serviço de Imunoalergologia Hospitais da Universidade de Coimbra, Praceta Mota Pinto, 3000-075 Coimbra, Portugal. E-mail:


Background and purpose: 

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.


Key words:

BCG, immunodeficiency, interferon-γ receptor 1 deficiency, tuberculosis, ZAP70 deficiency