Life-Threatening Pneumopathy and <i>U urealyticum</i> in a STAT3-Deficient Hyper-IgE Syndrome Patient.

Abstract

A deficiency in signal transducer and activator of transcription 3 (STAT3) is responsible for autosomal dominant hyperimmunoglobulin E syndrome, an immunodeficiency syndrome causing <i>Staphylococcus aureus</i>, <i>Streptococcus pneumonia</i>, <i>Haemophilus influenzae</i>, and, rarely, <i>Pseudomonas aeruginosa</i> and <i>Aspergillus sp</i> infections. Currently, intracellular pathogens are not targeted in the management of severe infections. The pathophysiologic mechanism of hyperimmunoglobulin E syndrome immunodeficiency has recently been linked to a disorder in the T helper 17 pathway and disruption of the interleukin -23/interleukin-17 axis. We report an unusual case of severe pleuropneumopathy by <i>Ureaplasma urealyticum</i> in a teenage girl with STAT3-deficient hyperimmunoglobulin E syndrome (STAT3 HIES). A previous case of severe lung infection by <i>Mycoplasma pneumoniae</i> has already been described in a STAT3-deficient patient, but <i>U urealyticum</i> has never been reported in patients with STAT3 HIES. After a review of the literature, it seems that the specific immunodeficiency pathway of STAT3 HIES exposes STAT3 HIES patients to <i>Ureaplasma</i> lung infections because the pathophysiology of STAT3 HIES and <i>Ureaplasma</i> is based on STAT3 and T helper 17 cells.