Prat Lola, Bouaziz Jean-David, Wallach Daniel, Vignon-Pennamen Marie-Dominique, Bagot Martine
Université Paris Diderot, Sorbonne Paris Cité; AP-HP, Paris, France; Service de Dermatologie et Service d'Anatomo-Pathologie, Hôpital Saint Louis, 1 Avenue Claude Vellefaux, 75010 Paris, France.
Université Paris Diderot, Sorbonne Paris Cité; AP-HP, Paris, France; Service de Dermatologie et Service d'Anatomo-Pathologie, Hôpital Saint Louis, 1 Avenue Claude Vellefaux, 75010 Paris, France.
Clin Dermatol. 2014 May-Jun;32(3):376-88. doi: 10.1016/j.clindermatol.2013.11.004. Epub 2013 Dec 3.
Neutrophilic dermatoses (ND) are inflammatory skin conditions characterized by a sterile infiltrate of normal polymorphonuclear leukocytes. The main clinical forms of ND include Sweet syndrome, pyoderma gangrenosum, erythema elevatum diutinum, subcorneal pustular dermatosis, and their atypical or transitional forms. ND are often idiopathic, but they may be associated with myeloid hematologic malignancies (Sweet syndrome), inflammatory bowel disease or rheumatoid arthritis (pyoderma gangrenosum), and monoclonal gammopathies (erythema elevatum diutinum, subcorneal pustular dermatosis). The possible infiltration of internal organs with neutrophils during the setting of ND underlies the concept of a neutrophilic systemic disease. ND may be seen as a polygenic autoinflammatory syndrome due to their frequent association with other autoinflammatory disorders (monogenic or polygenic) and the recent published efficacy of interleukin-1 blocking therapies in their management.
中性粒细胞性皮肤病（ND）是一种炎症性皮肤病，其特征是正常多形核白细胞的无菌浸润。ND的主要临床形式包括Sweet综合征、坏疽性脓皮病、持久性隆起性红斑、角层下脓疱性皮肤病及其非典型或过渡形式。ND通常为特发性，但可能与髓系血液系统恶性肿瘤（Sweet综合征）、炎症性肠病或类风湿性关节炎（坏疽性脓皮病）以及单克隆丙种球蛋白病（持久性隆起性红斑、角层下脓疱性皮肤病）相关。在ND发病过程中，中性粒细胞可能浸润内脏器官，这构成了中性粒细胞系统性疾病的概念基础。由于ND常与其他自身炎症性疾病（单基因或多基因）相关，且近期发表的研究表明白细胞介素-1阻断疗法在其治疗中有效，因此ND可被视为一种多基因自身炎症综合征。
