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NERDS综合征:另一例病例报告。

NERDS syndrome: an additional case report.

作者信息

Zenarola P, Melillo L, Bisceglia M, Carotenuto M, Lomuto M

机构信息

Department of Dermatology, Casa Sollievo della Sofferenza' Hospital, IRCCS, S. Giovanni Rotondo, Italy.

出版信息

Dermatology. 1995;191(2):133-8. doi: 10.1159/000246531.

Abstract

NERDS is an eosinophilic disorder recently described by Butterfield and characterized by an association of nodules, eosinophilia, rheumatism, dermatitis and swelling. We describe an additional case, the third, of this new eosinophilic syndrome. The cardinal features included joint and cutaneous manifestations with prominent para-articular nodules and rheumatism, xerosis, recurrent urticarial eruption with angioedema associated with tissue and peripheral blood eosinophilia. A drug-induced (diclofenac) allergic rash and lymphadenopathy appeared during the course of the illness. Persistent leukocytosis with a maximum of 65% of eosinophils, mostly exhibiting the hypodense phenotype (activation index), was always present. During the acute phase of the disease, flow-cytometric analysis of blood and bone marrow revealed proliferation of activated CD4+/OKDR+ T helper cells and CD25+/OKDR+ eosinophils.

摘要

NERDS是一种最近由巴特菲尔德描述的嗜酸性粒细胞疾病,其特征是结节、嗜酸性粒细胞增多、风湿症、皮炎和肿胀并存。我们描述了这一新的嗜酸性粒细胞综合征的又一病例,即第三例。主要特征包括关节和皮肤表现,伴有明显的关节旁结节和风湿症、皮肤干燥、伴有血管性水肿的复发性荨麻疹皮疹,以及组织和外周血嗜酸性粒细胞增多。病程中出现了药物性(双氯芬酸)过敏性皮疹和淋巴结病。始终存在持续性白细胞增多,嗜酸性粒细胞最多占65%,大多表现为低密度表型(激活指数)。在疾病急性期,对血液和骨髓进行的流式细胞术分析显示,活化的CD4+/OKDR+辅助性T细胞和CD25+/OKDR+嗜酸性粒细胞增殖。

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