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Review
. 2020 Jun;37(3):326-332.
doi: 10.5114/ada.2020.96142. Epub 2020 Jul 16.

Factors causing oral and skin pathological features in 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
Affiliations
Review

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

Karolina Gerreth et al. Postepy Dermatol Alergol. 2020 Jun.

Abstract

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: HIES); dermatological manifestation; environmental factor; hyperimmunoglobulin E syndrome (hyper-IgE syndrome; oral finding.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Discoloration of the lower lip reflecting a phototoxic reaction occurring during Voriconazole treatment as well as visible prominent pores on the facial skin
Figure 2
Figure 2
A high arched palate as well as midline sagittal fibrotic thickening covering an anterior part of the hard palate
Figure 3
Figure 3
Orthopantomograms taken when the girl was 5 years old
Figure 4
Figure 4
Orthopantomograms taken when the girl was 12 years old
See this image and copyright information in PMC

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