Chronological changes of skin eruptions toward cold abscess formation in hyper‐immunoglobulin E syndrome
Chronological changes of skin eruptions toward cold abscess formation in hyper‐immunoglobulin E syndrome
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高免疫球蛋白E综合征皮疹向冷脓肿形成的时间变化
DOI：
10.1111/1346-8138.15886
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发表时间：
2021
期刊：
影响因子：
--
通讯作者：
Aiba Setsuya
中科院分区：
文献类型：
--
作者：
Fukui Reimu;Hidaka Takanori;Terui Hitoshi;Rikiishi Takeshi;Sasahara Yoji;Kagimoto Yoshiko;Kusakari Yoshiyuki;Yamasaki Kenshi;Aiba Setsuya
亲爱的编辑：高免疫球蛋白E综合征(HIEs)是一种原发免疫缺陷疾病，于19661被首次描述为约伯综合征，并于1972年以血清Ig E水平升高为特征。2所谓的“寒性脓肿”是HIES的特征症状，尽管寒性脓肿的形成过程没有很好的文献记载。我们经历了一例HIES病例，从他出生起就密切监测了皮疹的时间变化。这名男婴是自然分娩，怀孕37周时出生体重3400克。他出生时全身都是脓包。考虑到母体产道内B组链球菌阳性，给予青霉素治疗，一周后脓肿好转。出生一个月后，面部复发播散性脓肿(图1a)。然后他发烧超过38摄氏度，皮损变成红皮性湿疹(图1b)。湿疹皮损在一周内转化为结痂(图1C)，然后在两周内自发改善(图1D)。他重复了每2-3周一次的脓疱形成、湿疹发作和皮损自然愈合的周期。97天脓疱性皮损的皮肤活检显示表皮棘皮松解，嗜酸性粒细胞和中性粒细胞渗入(图1E)。在第153天，他在枕区形成了一个巨大的寒性脓肿(图1f)。脓肿中检出甲氧西林敏感的金黄色葡萄球菌。D97的总IgE值为378IU/m L，D519及以后的总Ig E>30000 IU/m L。金黄色葡萄球菌肠毒素A和B在D148上的IGE分别为53.7和1.53IU/mL。MAST33在D519对念珠菌的IGE为8.21Lumicount。他在6个月大时被诊断为高免疫球蛋白E综合征，伴有STAT3 c.1909G和gt；A杂合突变(dSNP rs113994139，Sh2结构域Val637Met页)。他的父母和家人没有类似的症状。患者的皮肤损害总是在全身症状之前突然出现播散性小脓疹。脓疱性皮损随后转变为侵蚀性湿疹，如特应性皮炎，形成结痂，并愈合。皮损面积从面部到躯干逐渐扩大。皮疹每2-3周复发一次(补充资料中显示了从出生第0天到第153天的皮损图像)。当他在5个月大的时候出现冷性脓肿时，他已经对金黄色葡萄球菌产生了IgE，这表明他对反复接触金黄色葡萄球菌的敏感性增加了形成冷性脓肿的机会。由于HIES具有以下特点
Dear Editor, Hyper-immunoglobulin E syndrome (HIES) is a primary immunodeficiency disease, which was first described as Job's syndrome in 19661 and characterized by elevated serum IgE levels in 1972. 2 So called ‘cold abscess’ is a characteristic symptom of HIES though a process of cold abscess formation is not well documented. We have experienced a HIES case, in which chronological changes of skin eruptions were closely monitored from his birth. The boy was born by natural delivery with 3400 g of birth weight at 37 weeks of gestation. Pustules were already present all over his body at birth. Considering Group B Streptococcus-positive in the maternal birth canal, penicillin was given, and pustules improved in a week. A month after the birth, disseminated pustules recurred on the face (Figure 1a). He then had a fever over 38 C, and skin lesions became erythrodermic eczematous lesions (Figure 1b). The eczematous lesions turned to crusts in a week (Figure 1c), and then spontaneously improved in 2 weeks (Figure 1d). He repeated the cycle of pustule formation, eczematous eruption, and spontaneous healing of skin lesions every 2–3 weeks. A skin biopsy from a pustular lesion on day 97 revealed acantholysis in the epidermis and infiltration of eosinophils and neutrophils (Figure 1e). He developed a large cold abscess in the occipital region on day 153 (Figure 1f). Methicillin-sensitive Staphylococcus aureus were detected in the abscess. Total IgE values were 378 IU/mL on D97 and more than 30,000 IU/mL on D519 and later. IgE for S. aureus enterotoxin A and B were 53.7 and 1.53 IU/mL on D148, respectively. IgE for candida was 8.21 Lumicount by MAST33 on D519. He was diagnosed as a hyper-immunoglobulin E syndrome with a heterozygous mutation of STAT3 c. 1909G> A (dbSNP rs113994139, p. Val637Met in Sh2 domain) at 6 months of age. There was no similar symptom in his parents and family.The skin lesions of the patient always started with sudden onsets of disseminated small pustules preceding general symptoms. The pustular lesions then turned to erosive eczematous eruptions as atopic dermatitis, formed crusts, and healed. The area of the skin lesions was enlarged from the face to the trunk. The eruptions recurred every 2 to 3 weeks (The chronological skin lesion images from day 0 at his birth to day 153 are shown in the supplementary information). He had already developed IgE against S. aureus when he developed the cold abscess at 5 months of age, suggesting that his susceptibility to the repeated Staphylococci exposure increased a chance to form the cold abscess. Since HIES has features of
影响因子：
13.8
作者：
Olaiwan, Amani;Chandesris, Marie-Olivia;Bodemer, Christine
通讯作者：
Bodemer, Christine