Lorette G, Lebranchu Y, Legrand M F, Bardos P, Despert F, Grojean M F, Larrègue M
Ann Dermatol Venereol. 1984;111(1):39-46.
The first patient suffered from a very severe atopic dermatitis with intense pruritus and thickened skin. He had also recurrent infections, particularly related to Staphylococcus coagulase +, and axillary and inguinal lymphodermopathy. The use of tetracosactide given intramuscularly allowed controlling the evolution of his atopic dermatitis. After several months of treatment, the skin became less infiltrated, lymphodermopathy disappeared and no severe infection had happened. The second patient had a less severe atopic dermatitis and recurrent infections without any particular severity. Topical corticosteroids allowed to control the atopic dermatitis. These two patients had high levels of circulating IgE and an important deficiency of polymorphonuclear chemotaxis which was evaluated by migration through boyden room. Study of the family showed atopic manifestations in several members, but with lower levels of IgE. The most characteristic abnormality of this syndrome is the according to considerable increase of IgE. The deficit in polymorphonuclear chemotaxis may vary according to time and even become normal. The prognosis over long periods remains to be determined.
第一位患者患有非常严重的特应性皮炎，伴有剧烈瘙痒和皮肤增厚。他还反复感染，特别是与凝固酶阳性葡萄球菌有关，并有腋窝和腹股沟淋巴皮肤病。肌肉注射替可克肽可控制他特应性皮炎的病情发展。经过几个月的治疗，皮肤浸润减轻，淋巴皮肤病消失，未发生严重感染。第二位患者的特应性皮炎症状较轻，反复感染但无任何特别严重的情况。外用皮质类固醇可控制特应性皮炎。这两位患者的循环IgE水平较高，多形核白细胞趋化性明显缺陷，这是通过在博伊登小室中迁移来评估的。家族研究显示，几个家庭成员有特应性表现，但IgE水平较低。该综合征最具特征性的异常是IgE显著升高。多形核白细胞趋化性缺陷可能随时间变化，甚至恢复正常。长期预后仍有待确定。
