Neutrophilic Urticarial Dermatosis: A Window into Systemic Inflammation and Autoimmune Disorders

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.