eISSN: 2299-0046
ISSN: 1642-395X
Advances in Dermatology and Allergology/Postępy Dermatologii i Alergologii
Current issue Archive Manuscripts accepted About the journal Editorial board Journal's reviewers Abstracting and indexing Subscription Contact Instructions for authors
SCImago Journal & Country Rank
3/2020
vol. 37
 
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abstract:
Review paper

Factors causing oral and skin pathological featuresin the hyperimmunoglobulin E syndrome patient including the environmental component: a review of the literature and own experience

Karolina Gerreth
1
,
Aleksandra Szczawinska-Poplonyk
2
,
Zdzislawa Kycler
2
,
Zygmunt Adamski
3
,
Maria Borysewicz-Lewicka
4
,
Anna Breborowicz
2

1.
Department of Risk Group Dentistry, Chair of Paediatric Dentistry, Poznan University of Medical Sciences, Poznan, Poland
2.
Department of Paediatric Pneumonology, Allergology and Clinical Immunology, 3rd Chair of Paediatrics, Poznan University of Medical Sciences, Poznan, Poland
3.
Department of Dermatology, Poznan University of Medical Sciences, Poznan, Poland
4.
Chair and Department of Paediatric Dentistry, Poznan University of Medical Sciences, Poznan, Poland
Adv Dermatol Allergol 2020; XXXVII (3): 326-332
Online publish date: 2020/07/16
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The hyperimmunoglobulin E syndrome (HIES) is a rare multi-system disease with non-immunological as well as immunological abnormalities. The syndrome is characterized by a triad of the most distinctive symptoms, such as pneumonia with pneumatocele formation, recurring staphylococcal skin abscesses and a high serum concentration of IgE. Central mediators of immune responses such as STAT1 and STAT3 affect immune responses and contribute to changes of the skin microbiome which subsequently can amplify the defective immune response against microbial and fungal pathogens. Reactions related to an environmental factor, such as sun-induced skin changes, in individuals during long-term medication therapy have also been reported. The dermatological symptoms, oral status and other health problems of a hyperimmunoglobulin E syndrome paediatric patient are presented. HIES is of great importance to different professionals because sufferers require special preventive and therapeutic management from early infancy in order to avoid complications which can even prove to be life-saving for such patients.
keywords:

hyperimmunoglobulin E syndrome (hyper-IgE syndrome, HIES), environmental factor, dermatological manifestation, oral finding

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