Gahr M, Allgeier B, Speer C P
Monatsschr Kinderheilkd. 1987 Jun;135(6):329-35.
The Hyper-IgE-syndrome (Job-, Buckley-) is characterized by recurrent staphylococcal infections of the skin, the ears and the lungs, by an eczematoid dermatitis from early infancy on, and by extreme elevation of serum IgE. The inconstantly found decreased chemotaxis of the polymorphonuclear leukocytes seems to be a secondary sign of a so far unknown deficiency possibly of the T-cell mediated immunity. Thus, therapy is restricted to antibiotic and surgical treatment. Many patients have a typical coarse face and some involvement of the bones (osteoporosis, craniosynostosis).
高IgE综合征(乔布氏综合征、巴克利综合征)的特征为皮肤、耳部和肺部反复发生葡萄球菌感染,自婴儿早期起出现湿疹样皮炎,以及血清IgE极度升高。多形核白细胞趋化性降低的情况并非始终出现,这似乎是迄今未知的某种缺陷(可能是T细胞介导的免疫缺陷)的次要表现。因此,治疗仅限于抗生素治疗和手术治疗。许多患者面部特征典型且粗糙,部分患者骨骼受累(骨质疏松、颅缝早闭)。