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):1009-1022. doi: 10.1016/j.jaad.2017.11.063. 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 second article in this continuing medical education series reviews the epidemiology, clinical characteristics, histopathologic features, diagnosis, and management of pyoderma gangrenosum as well as bowel-associated dermatosis-arthritis syndrome and the arthritis-associated neutrophilic dermatoses rheumatoid neutrophilic dermatitis and adult Still disease.
中性粒细胞皮肤病是一组异质性的炎症性皮肤疾病，具有独特的临床特征，但组织病理学上均存在无菌性、以中性粒细胞为主的浸润。这些疾病相关的皮肤损害形态多样，这使得诊断具有挑战性。此外，需要进行全面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发病率，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。这个继续医学教育系列的第二篇文章回顾了坏疽性脓皮病、肠病相关性皮肤病关节炎综合征以及与关节炎相关的中性粒细胞皮肤病类风湿性中性粒细胞皮肤病和成人Still 病的流行病学、临床特征、组织病理学特征、诊断和治疗。
