Journal Article

<i>Paracoccidioides brasiliensis</i> Disseminated Disease in a Patient with Inherited Deficiency in the β1 Subunit of the Interleukin (IL)–12/IL-23 Receptor

Dewton de Moraes-Vasconcelos, Anete S. Grumach, Augusto Yamaguti, Maria Elisa B. Andrade, Claire Fieschi, Ludovic de Beaucoudrey, Jean-Laurent Casanova and Alberto J. S. Duarte

in Clinical Infectious Diseases

Published on behalf of Infectious Diseases Society of America

Volume 41, issue 4, pages 31-37
Published in print August 2005 | ISSN: 1058-4838
Published online August 2005 | e-ISSN: 1537-6591 | DOI: http://dx.doi.org/10.1086/432119
Paracoccidioides brasiliensis Disseminated Disease in a Patient with Inherited Deficiency in the β1 Subunit of the Interleukin (IL)–12/IL-23 Receptor

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Background. Paracoccidioides brasiliensis is a facultative intracellular dimorphic fungus that causes paracoccidioidomycosis (PCM), the most important deep mycosis in Latin America. Only a small percentage of individuals infected by P. brasiliensis develop clinical PCM, possibly in part because of genetically determined interindividual variability of host immunity. However, no primary immunodeficiency has ever been associated with PCM.

Methods. We describe the first patient, to our knowledge, with PCM and a well-defined primary immunodeficiency in the β1 subunit of the interleukin (IL)–12/IL-23 receptor, a disorder previously shown to be specifically associated with impaired interferon (IFN)–γ production, mycobacteriosis, and salmonellosis.

Results. Our patient had a childhood history of bacille Calmette-Guùrin disease and nontyphoid salmonellosis and, at the age of 20 years, presented to our clinic with a disseminated (acute) form of PCM. He responded well to antifungal treatment and is now doing well at 24 years of age.

Conclusions. This unique observation supports previous studies of PCM suggesting that IL-12, IL-23, and IFN-γ play an important role in protective immunity to P. brasiliensis. Tuberculosis and PCM are thus not only related clinically and pathologically, but also by their immunological pathogenesis. Our study further expands the spectrum of clinical manifestations of inherited defects of the IL-12/IL-23—IFN-γ axis. Patients with unexplained deep fungal infections, such as PCM, should be tested for defects in the IL-12/IL-23—IFN-γ axis.

Journal Article.  3359 words.  Illustrated.

Subjects: Infectious Diseases ; Immunology ; Public Health and Epidemiology ; Microbiology

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