Cai Xiaoxuan, Zheng Yihe, Yang Changyi, Xu Jiali, Fang Hong, Qiao Jianjun
Department of Dermatology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China.
Clin Rev Allergy Immunol. 2025 May 6;68(1):48. doi: 10.1007/s12016-025-09056-z.
Neutrophilic urticarial dermatosis (NUD) is a distinctive dermatological manifestation that is commonly associated with systemic autoinflammatory and autoimmune diseases. This review comprehensively explores NUD in the context of five major conditions: Schnitzler syndrome, Still's disease, cryopyrin-associated periodic syndrome, systemic lupus erythematosus, and VEXAS syndrome. For each condition, a detailed discussion of the underlying mechanisms, clinical presentations, diagnostic criteria, and treatment strategies is provided. In addition, cases exhibiting features similar to NUD are emphasized, with a comprehensive examination of the pathological characteristics, particularly focusing on neutrophilic epitheliotropism. This review underscores the significance of identifying NUD as a potential indicator of systemic autoimmune disorders and discusses the role of skin biopsy and laboratory tests in diagnosing the underlying etiology. Finally, a diagnostic framework for NUD is proposed, highlighting the importance of a multidisciplinary assessment to ascertain the underlying systemic condition responsible for the dermatological manifestations. The objective of this review is to enhance the comprehension of NUD, thereby facilitating early diagnosis and the implementation of targeted strategies for affected patients.
中性粒细胞性荨麻疹样皮肤病（NUD）是一种独特的皮肤表现，通常与系统性自身炎症性疾病和自身免疫性疾病相关。本综述在以下五种主要疾病的背景下全面探讨了NUD：施尼茨勒综合征、斯蒂尔病、冷吡啉相关周期性综合征、系统性红斑狼疮和VEXAS综合征。针对每种疾病，都详细讨论了其潜在机制、临床表现、诊断标准和治疗策略。此外，还强调了表现出与NUD相似特征的病例，并对病理特征进行了全面检查，特别关注中性粒细胞亲表皮现象。本综述强调了将NUD识别为系统性自身免疫性疾病潜在指标的重要性，并讨论了皮肤活检和实验室检查在诊断潜在病因中的作用。最后，提出了一个NUD的诊断框架，强调了多学科评估对于确定导致皮肤表现的潜在系统性疾病的重要性。本综述的目的是提高对NUD的理解，从而促进早期诊断并为受影响的患者实施针对性策略。
