Retained primary teeth in STAT3 hyper-IgE syndrome: early intervention in childhood is essential

Iris Meixner,Ellen D Renner,Ronny Hesse,Benedikt D Spielberger,Beate Hagl,Tim Niehues,Ekaterini Paschos,Carolin I Kröner,Gregor Dückers

doi:10.1186/s13023-020-01516-3

Abstract

BackgroundSTAT3 hyper-IgE syndrome (STAT3-HIES) is a rare primary immunodeficiency that clinically overlaps with atopic dermatitis. In addition to eczema, elevated serum-IgE, and recurrent infections, STAT3-HIES patients suffer from characteristic facies, midline defects, and retained primary teeth. To optimize dental management we assessed the development of dentition and the long-term outcomes of dental treatment in 13 molecularly defined STAT3-HIES patients using questionnaires, radiographs, and dental investigations.ResultsPrimary tooth eruption was unremarkable in all STAT3-HIES patients evaluated. Primary tooth exfoliation and permanent tooth eruption was delayed in 83% of patients due to unresorbed tooth roots. A complex orthodontic treatment was needed for one patient receiving delayed extraction of primary molars and canines. Permanent teeth erupted spontaneously in all patients receiving primary teeth extraction of retained primary teeth during average physiologic exfoliation time.ConclusionsThe association of STAT3-HIES with retained primary teeth is important knowledge for dentists and physicians as timely extraction of retained primary teeth prevents dental complications. To enable spontaneous eruption of permanent teeth in children with STAT3-HIES, we recommend extracting retained primary incisors when the patient is not older than 9 years of age and retained primary canines and molars when the patient is not older than 13 years of age, after having confirmed the presence of the permanent successor teeth by radiograph.

Highlights

signal transducer and activator of transcription 3 (STAT3) hyper-IgE syndrome (STAT3-HIES) is a rare primary immunodeficiency that clinically overlaps with atopic dermatitis
Dental history of two STAT3-HIES patients The dental histories of two exemplary patients show the outcome of different treatment strategies: In patient #4 the primary teeth were all extracted at the average exfoliation time, while patient #3 received some tooth extractions later in life
In this report, we focused on dental and oral findings in STAT3-HIES patients to raise awareness of an optimized dental treatment to prevent dental complications in adolescence

Summary

Introduction

STAT3 hyper-IgE syndrome (STAT3-HIES) is a rare primary immunodeficiency that clinically overlaps with atopic dermatitis. In addition to eczema, elevated serum-IgE, and recurrent infections, STAT3-HIES patients suffer from characteristic facies, midline defects, and retained primary teeth. STAT3 hyper-IgE syndrome is a rare primary immunodeficiency associated with eczema, recurrent infections, and high serum IgE levels [1, 2]. Non-immunologic findings such as scoliosis, pathologic fractures, characteristic facies, a high arched palate, midline defects, and retained primary teeth have been reported in STAT3-HIES [1, 2, 13,14,15,16]. Ankylosis of primary teeth and impaction, abnormal position, and late eruption of successor teeth may likewise cause retention of permanent teeth [19]

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