eISSN: 1644-4124
ISSN: 1426-3912
Central European Journal of Immunology
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SCImago Journal & Country Rank
3/2023
vol. 48
 
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abstract:
Clinical immunology

Intraoral and maxillofacial abnormalities in patients with autosomal dominant hyper-IgE syndrome

Ildikó Tar
1
,
Márta Szegedi
1
,
Ewa Krasuska-Sławińska
2
,
Edyta Heropolitańska-Pliszka
3
,
Ewa A. Bernatowska
3
,
Elif Öncü
4
,
Sevgi Keles
5
,
Sukru N. Guner
5
,
Ismail Reisli
5
,
Nevena Gesheva
6
,
Elissaveta Naumova
6
,
Lydie Izakovicova-Holla
7
,
Jiri Litzman
8
,
Igor Savchak
9
,
Larysa Kostyuchenko
9
,
Melinda Erdős
10

  1. Faculty of Dentistry, University of Debrecen, Debrecen, Hungary
  2. Dental Surgical Clinic for Children, Children’s Memorial Health Institute, Warsaw, Poland
  3. Department of Immunology, Children’s Memorial Health Institute, Warsaw, Poland
  4. Department of Periodontology, Lokman Hekim University, Ankara, Turkey
  5. Division of Pediatric Allergy and Immunology, Necmettin Erbakan University, Konya, Turkey
  6. Department of Clinical Immunology and Stem Cell Bank, University Hospital “Aleksandrovska”, Sofia, Bulgaria
  7. Department of Stomatology, St Anne’s University Hospital, Faculty of Medicine, Masaryk University, Brno, Czech Republic
  8. Department of Clinical Immunology and Allergology, St Anne’s University Hospital, Faculty of Medicine, Masaryk University, Brno, Czech Republic
  9. Department of Pediatric Immunology and Rheumatology, Western Ukrainian Specialized Children’s Medical Center, Lviv, Ukraine
  10. J Project Education and Research Network, Debrecen, Hungary
Cent Eur J Immunol 2023; 48 (3): 228-236
Online publish date: 2023/09/05
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Autosomal dominant hyper-IgE syndrome (AD-HIES) is an inborn error of immunity (IEI) caused by a dominant-negative mutation in the signal transducer and activator of transcription 3 (STAT 3). This disease is characterized by chronic eczematoid dermatitis, recurrent staphylococcal skin abscesses, pneumonia, pneumatoceles, and extremely high serum IgE levels. Loss-of-function STAT3 mutations may also result in distinct non-immunologic features such as dental, facial, skeletal, and vascular abnormalities, central nervous system malformations and an increased risk for bone fractures. Prophylactic treatment of Candida infections and prophylactic antimicrobial therapy for staphylococcal skin infections and sinopulmonary infections are essential. An awareness of the oral and maxillofacial features of HIES may facilitate early diagnosis with genetic counselling and may improve future patient care. This study describes oral, dental, and maxillofacial manifestations in 14 patients with genetically defined AD-HIES. We also review the literature and propose recommendations for the complex care of patients with this rare primary immunodeficiency.
keywords:

dentist, hyper-IgE syndrome, maxillofacial, intraoral

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