Weiss Emma H, Ko Christine J, Leung Thomas H, Micheletti Robert G, Mostaghimi Arash, Ramachandran Sarika M, Rosenbach Misha, Nelson Caroline A
Department of Dermatology, Yale School of Medicine, New Haven, CT USA.
Department of Dermatology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA USA.
Curr Dermatol Rep. 2022;11(2):89-102. doi: 10.1007/s13671-022-00355-8. Epub 2022 Mar 16.
Neutrophilic dermatoses are defined by the presence of a sterile neutrophilic infiltrate on histopathology. This review focuses on the pathogenesis, epidemiology, clinicopathological features, diagnosis, and management of four disorders: Sweet syndrome, pyoderma gangrenosum, Behçet syndrome, and neutrophilic eccrine hidradenitis.
Recent studies have provided insight into the complex pathogenesis of neutrophilic dermatoses. Evidence supports an intricate interplay of abnormal neutrophil function and inflammasome activation, malignant transformation into dermal infiltrating neutrophils, and genetic predisposition.
Neutrophilic dermatoses have diverse cutaneous and extracutaneous manifestations and may be associated with significant morbidity and mortality. Common underlying associations include infectious, inflammatory, and neoplastic disorders, as well as drug reactions. Emerging diagnostic and therapeutic frameworks identify an expanding role for biologic and targeted anti-inflammatory therapies.
嗜中性皮病通过组织病理学上无菌性嗜中性粒细胞浸润来定义。本综述重点关注四种疾病的发病机制、流行病学、临床病理特征、诊断和管理:Sweet综合征、坏疽性脓皮病、白塞病和嗜中性小汗腺汗管炎。
最近的研究为嗜中性皮病的复杂发病机制提供了见解。有证据支持异常嗜中性粒细胞功能与炎性小体激活、向真皮浸润嗜中性粒细胞的恶性转化以及遗传易感性之间的复杂相互作用。
嗜中性皮病有多种皮肤和皮肤外表现,可能与显著的发病率和死亡率相关。常见的潜在关联包括感染性、炎症性和肿瘤性疾病以及药物反应。新兴的诊断和治疗框架确定了生物和靶向抗炎疗法日益扩大的作用。