Hyper-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.

高IgE综合征（HIES）患者可能具有在严重特应性皮炎（SAD）中观察到的许多特征，这为医生带来了诊断难题。确定可以区分两种疾病的临床和实验室标志物可以提供早期诊断和治疗。我们分析了患有早发性SAD的患者（DOCK8缺乏症：14，STAT3-HIES：10，SAD：10）。与SAD患者相比，HIES中复发性上呼吸道感染和肺炎的发生率明显更高。STAT3-HIES更容易预测特征性的面部外观，保留的乳牙，皮肤脓肿，新生儿皮疹和肺膨出，而DOCK8缺乏症中粘膜皮肤念珠菌病和疱疹感染常见，而SAD组则不常见。DOCK8缺乏症患者的CD3 ⁺和CD4 ⁺较低具有这种HIES形式的衰老表型的T细胞。与SAD患者相比，DOCK8缺乏症和STAT3-HIES患者均显示出减少的开关记忆B细胞。这些临床和实验室标志物有助于区分HIES和SAD患者。