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New research · Allergy & Immunology
The British journal of dermatology · 23h
Cohort studyThe British journal of dermatology · 2026

Eczema in inborn errors of immunity with atopy: frequency, timing, phenotypic clustering.

Hélène Aubert, Nizar Mahlaoui, Sébastien Barbarot … Caroline Thomas
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Allergy & ImmunologyCohort study

Over half of patients with inborn errors of immunity with atopy have eczema.

Eczema in inborn errors of immunity with atopy: frequency, timing, phenotypic clustering.

Hélène Aubert et al. · The British journal of dermatology · 2026
Background

Inborn errors of immunity with atopy (IEIs-A) are an IEI subgroup with eczema as a prominent clinical feature that can mimic atopic dermatitis (AD).

Purpose

To characterize eczema frequency, timing and phenotypes in IEIs-A patients, and explore eczema's relationship with other clinical IEIs-A manifestations.

Methods

This retrospective study used data from the French national IEI (CEREDIH) Registry database, focusing on patients with genetically defined IEIs-A followed in three large CEREDIH-participating centers and available clinical data.

n = 373 IEIs-A patients
50.9%
Results
over half of patients with inherited immune system problems and allergies had eczema
n = 373 IEIs-A patients
More results

All dedicator of cytokinesis-8 (DOCK8)-deficient patients were affected, followed by Forkhead box-P3 (FOXP3)-deficient patients (73.3%), and those with signal transducer and activator of transcription-1-loss-of-function (STAT3-LoF; 74.6%).

Onset was usually early (median: 4 months).

More results

Eczema frequency and clinical presentation varied markedly across IEIs-A genotypes; four phenotypic clusters were identified, with particular localizations and severe pruritus being strongly associated with pathogenic STAT3 or DOCK8 variants.

“
Conclusion

Eczema is a common, early IEIs-A feature, often preceding infections by years. While 38.4% of IEIs-A patients with eczema fulfilled the UK Working Party diagnostic criteria for AD, some clinical pictures may differ from that of classic AD.

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