Disseminated Histoplasmosis with Liver Involvement in a Job’s Syndrome Patient

Autosomal dominant hyper-IgE syndrome (AD-HIES), also known as Job’s syndrome, is caused by dominant negative mutations in signal transducer and activator of transcription 3 (STAT3), the key transcriptional factor for many cytokines and growth factors that are essential for the homeostasis of immune and nonimmune systems. Patients with Job’s syndrome have reduced Th17 cell numbers and functions, impaired neutrophil functions and adaptive immunity, which compromise their abilities to overcome fungal infections. While healthy individuals are asymptomatic with histoplasmosis, immunocompromised patients are susceptible to invasive histoplasmosis. We present the first case of disseminated histoplasmosis with liver involvement in a Job’s syndrome patient.