Paslin D, Norman M E
Arch Dermatol. 1977 Jun;113(6):801-5.
A 22-year-old white woman with Job's syndrome was found to have atopic dermatitis and impaired neutrophil chemotaxis in vitro. Major clinical features of Job's syndrome included large, "cold" and recurrent staphylococcal abscesses, and intermittent bacterial and yeast infections. Evidence for atopic disease included infantile eczema progressing to flexural dermatitis, a family history of atopy, positive immediate hypersensitivity skin tests, and hyperimmunoglobulinemia E. Defective erythema responses to histamine, methyl niacinate, and methacholine (Mecholyl) chloride may explain the lack of redness, heat, or pain signalling the development of abscesses (hence the term "cold"). Impaired chemotaxis was probably due to an intrinsic neutrophil defect since patient's serum generated normal amounts of chemotactic factors and did not contain an inhibitor of neutrophil chemotaxis. A delay in neutrophil exudation in vivo may explain the abscess formations and the atopic diathesis may explain the absence of clinical signs of inflammation that have been described in this and other patients with Job's syndrome.
一名患有乔布综合征的22岁白人女性被发现患有特应性皮炎，且体外中性粒细胞趋化功能受损。乔布综合征的主要临床特征包括巨大、“冷性”且反复发作的葡萄球菌脓肿，以及间歇性的细菌和酵母菌感染。特应性疾病的证据包括婴儿湿疹发展为屈侧皮炎、特应性家族史、速发型超敏反应皮肤试验阳性以及高免疫球蛋白E血症。对组胺、烟酸甲酯和氯化乙酰甲胆碱（美加明）的红斑反应缺陷可能解释了脓肿形成时缺乏发红、发热或疼痛信号（因此有“冷性”一词）。趋化功能受损可能是由于中性粒细胞内在缺陷，因为患者血清产生正常量的趋化因子，且不含有中性粒细胞趋化抑制剂。体内中性粒细胞渗出延迟可能解释了脓肿的形成，而特应性素质可能解释了在该患者及其他乔布综合征患者中所描述的炎症临床体征的缺失。
