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Öğe Atypical Localization of Eczema Discriminates DOCK8 or STAT3 Deficiencies from Atopic Dermatitis(Springer/Plenum Publishers, 2023) Kasap, Nurhan; Kara, Altan; Celik, Velat; Eltan, Sevgi Bilgic; Haci, Idil Akay; Kose, Hulya; Aygun, AysePurposeAutosomal 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.Öğe A set of clinical and laboratory markers differentiates hyper-IgE syndrome from severe atopic dermatitis(Academic Press Inc Elsevier Science, 2021) Kasap, Nurhan; Celik, Velat; Isik, Sakine; Cennetoglu, Pakize; Kiykim, Ayca; Eltan, Sevgi Bilgic; Nain, ErcanHyper-IgE syndrome (HIES) patients may share many features observed in severe atopic dermatitis (SAD), making a diagnostic dilemma for physicians. Determining clinical and laboratory markers that distinguish both disorders could provide early diagnosis and treatment. We analyzed patients (DOCK8 deficiency:14, STAT3-HIES:10, SAD:10) with early-onset SAD. Recurrent upper respiratory tract infection and pneumonia were significantly frequent in HIES than SAD patients. Characteristic facial appearance, retained primary teeth, skin abscess, newborn rash, and pneumatocele were more predictable for STAT3-HIES, while mucocutaneous candidiasis and Herpes infection were common in DOCK8 deficiency, which were unusual in SAD group. DOCK8-deficient patients had lower CD3(+) and CD4(+)T cells with a senescent phenotype that unique for this form of HIES. Both DOCK8 deficiency and STAT3-HIES patients exhibited reduced switched memory B cells compared to the SAD patients. These clinical and laboratory markers are helpful to differentiate HIES from SAD patients.