Primary Invasive Cutaneous Fusariosis in Patients with STAT3 Hyper-IgE Syndrome

Successful Use of Fosmanogepix for Treatment of Rare Highly Resistant Cutaneous Fusariosis in a Pediatric Patient With STAT3 Hyper-Immunoglobulin E Syndrome and End-Stage Kidney Disease

“…Primary invasive cutaneous Fusarium infections have been described in STAT3 AD-HIES patients [ 1 , 23 ] who have impaired interleukin (IL)-17 expression and Th17 differentiation leading to defects in the immunological skin barrier. As opposed to the disseminated disease seen in neutropenic patients, the development of chronic, nonsystemic Fusarium skin disease in STAT3 AD-HIES is hypothesized to be due to impaired IL-17-dependent antimicrobial peptide generation at the keratinocyte level, resulting in infection confined to the skin [ 1 ]. In some types of primary immunodeficiencies, haploidentical HSCT followed by kidney transplant from the same parent donor has eliminated the need for post-SOT immunosuppression [ 24 ].…”

“…The patient is a 14-year-old Hispanic male diagnosed with signal transducer and activator of transcription 3 (STAT3) autosomal dominant hyper-immunoglobulin E syndrome (STAT3 AD-HIES) primary immunodeficiency in early childhood, whose prior course has been published [ 1 , 2 ]. At age 3 years of age, he developed a 3 × 3-cm plaque on his right forearm after a dog bite, later diagnosed as Fusarium solani species complex (FSSC) infection.…”

Human–Fungal Pathogen Interactions from the Perspective of Immunoproteomics Analyses

“…Invasive fungal diseases were reported in patients diagnosed with STAT3-mutated hyper-IgE syndrome. The fungi isolated from affected patients included Candida [ 33 , 34 ], Aspergillus [ 35 , 36 , 37 ], Histoplasma [ 35 ], Talaromyces [ 21 ], Coccidioides [ 38 ], Cryptococcus [ 35 , 38 ], and Fusarium species [ 39 ].…”