Department of Dermatology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.
Department of Dermatology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.
J Am Acad Dermatol. 2018 Dec;79(6):987-1006. doi: 10.1016/j.jaad.2017.11.064. Epub 2018 Apr 11.
Neutrophilic dermatoses are a heterogeneous group of inflammatory skin disorders that present with unique clinical features but are unified by the presence of a sterile, predominantly neutrophilic infiltrate on histopathology. The morphology of cutaneous lesions associated with these disorders is heterogeneous, which renders diagnosis challenging. Moreover, a thorough evaluation is required to exclude diseases that mimic these disorders and to diagnose potential associated infectious, inflammatory, and neoplastic processes. While some neutrophilic dermatoses may resolve spontaneously, most require treatment to achieve remission. Delays in diagnosis and treatment can lead to significant patient morbidity and even mortality. Therapeutic modalities range from systemic corticosteroids to novel biologic agents, and the treatment literature is rapidly expanding. The first article in this continuing medical education series explores the pathogenesis of neutrophilic dermatoses and reviews the epidemiology, clinical and histopathologic features, diagnosis, and management of Sweet syndrome, neutrophilic eccrine hidradenitis, and Behçet disease.
中性粒细胞皮肤病是一组异质性炎症性皮肤疾病,具有独特的临床特征,但通过组织病理学上存在无菌、主要为中性粒细胞浸润而统一。与这些疾病相关的皮肤病变的形态是多种多样的,这使得诊断具有挑战性。此外,需要进行彻底的评估以排除模仿这些疾病的疾病,并诊断潜在的相关感染、炎症和肿瘤过程。虽然一些中性粒细胞皮肤病可能会自行缓解,但大多数需要治疗才能缓解。诊断和治疗的延迟会导致患者出现严重的疾病甚至死亡。治疗方法从全身皮质类固醇到新型生物制剂不等,治疗文献正在迅速扩展。本继续教育系列的第一篇文章探讨了中性粒细胞皮肤病的发病机制,并回顾了Sweet 综合征、中性粒细胞性大汗腺炎和 Behçet 病的流行病学、临床和组织病理学特征、诊断和治疗。