Atypical Localization of Eczema Discriminates DOCK8 or STAT3 Deficiencies from Atopic Dermatitis

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dc.contributor.authorKasap, Nurhan
dc.contributor.authorKara, Altan
dc.contributor.authorCelik, Velat
dc.contributor.authorEltan, Sevgi Bilgic
dc.contributor.authorHaci, Idil Akay
dc.contributor.authorKose, Hulya
dc.contributor.authorAygun, Ayse
dc.date.accessioned2024-02-23T13:56:15Z
dc.date.available2024-02-23T13:56:15Z
dc.date.issued2023
dc.departmentNEÜen_US
dc.description.abstractPurposeAutosomal recessive dedicator of cytokinesis 8 (DOCK8(-/-)) and autosomal dominant signal transducer and activator of transcription 3 (STAT3(-/+)) deficiencies are inborn errors of immunity (IEI) disorders present with the classic features of eczema and create a dilemma during differentiation from atopic dermatitis (AD). Therefore, an appropriate approach is required for eczema to diagnose DOCK8(-/-) and STAT3(-/+) early. Here, we described a set of clinical and immunological variables, including atypical AD localizations and lymphocyte subsets, to differentiate DOCK8(-/-) or STAT3(-/+) from AD.MethodsThis multicenter study involved 100 patients with DOCK8(-/-) and STAT3(-/+) and moderate/severe AD. We recruited disease manifestations, including detailed localizations of eczema, infections, and allergy. Principle component analysis (PCA) was used to discriminate DOCK8(-/-) or STAT3(-/+) from AD.ResultsThere were 43 patients with DOCK8(-/-), 23 with STAT3(-/+), and 34 with AD. Pneumonia, severe infections, mucocutaneous candidiasis, and skin abscesses were commonly observed in DOCK8 and STAT3 deficiencies. Atypical skin involvement with neonatal rash, retro auricular, axillary, sacral, and genital eczema discriminate DOCK8(-/-) and STAT3(-/+) from AD with high specificity ranges between 73.5 and 94.1% and positive predictive index ranges between 55 and 93.1%. Together with using absolute numbers of CD3(+), CD4(+), and CD8(+) T cells, the combined clinical and laboratory features showed perfect differentiation between DOCK8(-/-) or STAT3(-/+) and AD via PCA.ConclusionsThe described features can be easily implemented by physicians providing early diagnosis of DOCK8 and STAT3 deficiencies.en_US
dc.description.sponsorshipScientific and Technological Research Council of Turkey [318S202]en_US
dc.description.sponsorshipThis work was supported by the Scientific and Technological Research Council of Turkey to S.B. (318S202).en_US
dc.identifier.doi10.1007/s10875-023-01554-z
dc.identifier.issn0271-9142
dc.identifier.issn1573-2592
dc.identifier.pmid37507632en_US
dc.identifier.scopus2-s2.0-85166015909en_US
dc.identifier.scopusqualityQ1en_US
dc.identifier.urihttps://doi.org/10.1007/s10875-023-01554-z
dc.identifier.urihttps://hdl.handle.net/20.500.12452/11147
dc.identifier.wosWOS:001039821200001en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherSpringer/Plenum Publishersen_US
dc.relation.ispartofJournal Of Clinical Immunologyen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.subjectDock8 Deficiencyen_US
dc.subjectStat3 Deficiencyen_US
dc.subjectAtopic Dermatitisen_US
dc.subjectEczema Localizationsen_US
dc.titleAtypical Localization of Eczema Discriminates DOCK8 or STAT3 Deficiencies from Atopic Dermatitisen_US
dc.typeArticleen_US

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