Abstract：
Schnitzler syndrome (SchS) is a rare autoinflammatory disease characterized by bone pain, recurrent fever, leukocytosis, and elevated C-reactive protein, along with an urticaria-like rash and monoclonal immunoglobulin (Ig)M or IgG gammopathy. Notably, the condition is distinguished by a relatively persistent recurrent urticarial-like rash. Histopathological features observed in the skin comprise diffuse neutrophil infiltration into the dermis, absence of dermal edema, and vascular wall degeneration, all of which classify SchS as a neutrophilic urticarial dermatosis (NUD). Accumulated histological data from skin biopsies of patients with NUD have revealed a sensitive histopathological marker for NUD, acknowledged as neutrophilic epitheliotropism, which has been proposed as reflecting an autoinflammatory condition. In this report, we present three SchS patients: two men (ages 55 and 68) and a woman (age 75), all displaying neutrophilic epitheliotropism in their skin biopsy specimens. Additionally, a review of eight previously reported SchS cases in Japan identified neutrophilic epithliotropism in five cases. These findings suggest that the inclination of neutrophils toward the epithelial tissue could aid in confirming diagnoses of NUD in most cases that need to be differentiated from conventional urticaria. Consequently, we emphasize that acknowledging neutrophilic epithelial predilection as a hallmark of NUD is critical for expediting early diagnosis and appropriate treatment for SchS.
摘要：
Schnitzler综合征（SchS）是一种罕见的以骨痛为特征的自身炎症性疾病，反复发烧,白细胞增多,C反应蛋白升高,伴随荨麻疹样皮疹和单克隆免疫球蛋白（Ig）M或IgG丙种球蛋白病。值得注意的是,这种情况的特点是相对持续的复发性荨麻疹样皮疹。在皮肤中观察到的组织病理学特征包括弥漫性中性粒细胞浸润到真皮中，没有真皮水肿，血管壁退化，所有这些都将SchS分类为嗜中性荨麻疹皮肤病（NUD）。NUD患者皮肤活检的累积组织学数据揭示了NUD的敏感组织病理学标志物，被认为是嗜中性粒细胞上皮性，这已被认为反映了自身炎症。在这份报告中,我们介绍了三名SchS患者：两名男性（55岁和68岁）和一名女性（75岁），在他们的皮肤活检标本中都显示嗜中性上皮性。此外,对日本先前报道的8例SchS病例进行的回顾发现,5例患者存在嗜中性粒细胞性上皮性倾向.这些发现表明，在大多数需要与常规荨麻疹区分开的情况下，中性粒细胞向上皮组织的倾斜可以帮助确认NUD的诊断。因此,我们强调,承认嗜中性上皮好发是NUD的标志,对于加快SchS的早期诊断和适当治疗至关重要.
