Dental/oral complications of inborn errors of immunity (IEI)

Disorder	Abnormal tooth development	Infections	Inflammatory issues
Ataxia-telangiectasia (AT)		Candidiasis Viral stomatitis
C3 deficiency		Abscess
CD40/CD40 ligand deficiency		Abscess Candidiasis Gingivitis Periodontitis Viral stomatitis	Ulcerative stomatitis
Cherubism	Abnormal eruption Abnormal shedding
Chronic atypical neutrophilic dermatitis with lipodystrophy and elevated temperature (CANDLE) syndrome		Periodontitis
Chronic granulomatous disease (CGD)		Gingivitis Periodontitis
Chronic mucocutaneous candidiasis (CMCC)		Candidiasis
Common variable immunodeficiency (CVID)/Good syndrome		Abscess Periodontitis Viral stomatitis	Leukoplakia Aphthous stomatitis
CRAC channel pathway defects (ORAI1, STIM1, ITPR3)	Enamel defects Hypodontia^*/conical teeth (ITPR3)
DiGeorge syndrome (DGS)	Enamel hypomineralization Abnormal eruption	Caries
Dyskeratosis congenita (DC)	Taurodontism^¶	Periodontitis	Leukoplakia
Ectodermal dysplasia with immunodeficiency due to hypomorphic NEMO variants	Hypodontia^* Conical teeth	Viral stomatitis
Familial Mediterranean fever (FMF)			Ulcerative stomatitis
Hyperimmunoglobulin D syndrome (HIDS)			Ulcerative stomatitis
Hyperimmunoglobulin E syndrome (HIES)	Abnormal eruption Abnormal shedding	Candidiasis Gingivitis Periodontitis
IgG subclass deficiency		Viral stomatitis
Leukocyte-adhesion deficiency (LAD)		Periodontitis
Major histocompatibility complex (MHC) deficiency		Viral stomatitis
Neutropenia		Abscess Candidiasis Gingivitis Periodontitis
Periodic fever with aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome			Aphthous stomatitis
Schimke syndrome	Hypodontia^* Microdontia
Selective IgA deficiency (sIgAD)		Candidiasis Viral stomatitis	Aphthous stomatitis
Severe combined immunodeficiency (SCID)		Candidiasis Viral stomatitis	Ulcerative stomatitis
Sterile alpha motif domain and HD domain-containing protein (SAMHD1) deficiency			Ulcerative stomatitis
Transient hypogammaglobulinemia		Candidiasis
Wiskott-Aldrich syndrome (WAS)^Δ			Ulcerative stomatitis
X-linked agammaglobulinemia (XLA)		Candidiasis Gingivitis Viral stomatitis

CRAC: Ca²⁺ release-activated Ca²⁺; IgA: immunoglobulin A; IgG: immunoglobulin G; ITPR3: inositol 1,4,5-trisphosphate receptor type 3; NEMO: nuclear factor-kappa-B essential modulator; ORAI1: ORAI calcium release-activated calcium modulator 1; STIM1: stromal interaction molecule 1.

* The failure of up to 5 permanent teeth to develop (also called tooth agenesis).

¶ An abnormal tooth shape in which the roots are reduced in size and the body of the tooth is enlarged, most often affecting molars.

Δ Also bleeding and petechiae.

Adapted from: Peacock ME, Arce RM, Cutler CW. Periodontal and other oral manifestations of immunodeficiency diseases. Oral Dis 2017; 23:866.

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Dental/oral complications of inborn errors of immunity (IEI)

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