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Erschienen in: Journal of Clinical Immunology 3/2023

12.12.2022 | Original Article

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

verfasst von: Salam Abbara, Alexandra F. Freeman, Jérémie F. Cohen, Stéphanie Leclerc-Mercier, Lauren Sanchez, Joel Schlatter, Salvatore Cisternino, Ruth Parker, Edward W. Cowen, Claire Rouzaud, Marie Elisabeth Bougnoux, Fanny Lanternier, Michail S. Lionakis, Olivier Lortholary

Erschienen in: Journal of Clinical Immunology | Ausgabe 3/2023

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Abstract

Dominant negative (DN) mutations in signal transducer and activator of transcription 3 (STAT3) are known to cause hyper-IgE syndrome, a rare primary immunodeficiency. STAT3 DN patients are prone to develop fungal infections, including chronic mucocutaneous candidiasis due to impaired IL-17-mediated immunity, and pulmonary aspergillosis. Despite having preserved phagocyte functions, STAT3 DN patients present connective tissue abnormalities and a defect in the immunological skin barrier. Fusarium species are ubiquitous molds, whose potential to infect humans depends on the host’s innate and cellular immune status. Our aim was to describe four STAT3 DN patients with fusariosis confined to the skin. Medical records were reviewed and summarized. Four patients, aged 4, 11, 30, and 33 years, presented with chronic skin lesions which started in the extremities. Two patients had remote lesions, and none had systemic involvement. Skin biopsies showed mycelial threads with deep inflammatory—occasionally granulomatous—infiltrates, reaching the dermis; cultures grew Fusarium solani. Response to treatment was heterogeneous, often requiring multimodal therapies, including topical antifungal preparations. In this work, we describe primary invasive cutaneous fusariosis as a syndromic entity in four STAT3 DN patients.
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Metadaten
Titel
Primary Invasive Cutaneous Fusariosis in Patients with STAT3 Hyper-IgE Syndrome
verfasst von
Salam Abbara
Alexandra F. Freeman
Jérémie F. Cohen
Stéphanie Leclerc-Mercier
Lauren Sanchez
Joel Schlatter
Salvatore Cisternino
Ruth Parker
Edward W. Cowen
Claire Rouzaud
Marie Elisabeth Bougnoux
Fanny Lanternier
Michail S. Lionakis
Olivier Lortholary
Publikationsdatum
12.12.2022
Verlag
Springer US
Erschienen in
Journal of Clinical Immunology / Ausgabe 3/2023
Print ISSN: 0271-9142
Elektronische ISSN: 1573-2592
DOI
https://doi.org/10.1007/s10875-022-01404-4

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