Bazex Jacques, Bayle Paule
Service de Dermatologie, Allergologie, Vénéréologie, Hôpital Purpan. TSA 40031, 31059 Toulouse.
Bull Acad Natl Med. 2005 May;189(5):917-31; discussion 931-3.
Neutrophilic diseases are a group of disorders--mainly dermatoses--in which polymorphonuclear neutrophils accumulate in the absence of an identified pathogen. Such neutrophil infiltration can lead to a variety of clinical manifestations, including inflammatory disorders of the digestive tract, and hematological and rheumatological disorders; monoclonal or polyclonal circulating IgA levels are elevated, and the disorders are sensitive to drugs acting on neutrophils. Other disorders associated with neutrophil accumulation do not share these criteria. Non neutrophilic manifestations may also be present, and determine the prognosis. Neutrophilic dermatoses are often chronic and relapsing-remitting. Spontaneous regression occurs in some cases. It is unclear whether these pleomorphic disorders should be grouped together under a single heading In addition, the causes of the neutrophil accumulation are unknown. During the past five years, we have managed 31 patients with neutrophilic dermatoses. Here we describe our findings and review previous reports.
嗜中性粒细胞性疾病是一组疾病——主要是皮肤病——在这些疾病中,多形核嗜中性粒细胞在没有明确病原体的情况下积聚。这种嗜中性粒细胞浸润可导致多种临床表现,包括消化道炎症性疾病、血液学和风湿病疾病;单克隆或多克隆循环IgA水平升高,且这些疾病对作用于嗜中性粒细胞的药物敏感。与嗜中性粒细胞积聚相关的其他疾病并不具备这些标准。也可能存在非嗜中性粒细胞性表现,并决定预后。嗜中性粒细胞性皮肤病通常为慢性且呈复发-缓解型。在某些情况下会发生自发消退。尚不清楚这些多形性疾病是否应归为一类。此外,嗜中性粒细胞积聚的原因尚不清楚。在过去五年中,我们诊治了31例嗜中性粒细胞性皮肤病患者。在此我们描述我们的发现并回顾既往报告。