Objectives: Familial Mediterranean fever (FMF) is the most frequent monogenic autoinflammatory disease. It is associated with MEFV mutations. Its main features are recurrent episodes of fever and serositis. Patients can display dermatological manifestations such as erysipelas-like erythema, generally considered as a neutrophilic dermatosis (ND). It has been suggested that FMF can be associated with other types of ND. Our aim was to perform a systematic review of the literature to assess the link between ND and FMF.

Method: A systematic review of the literature was performed using MEDLINE from 1946 to 2018. Three independent investigators identified reports of non-erysipelas-like erythema neutrophilic dermatosis (NEND) associated with FMF, selected the criteria to establish the diagnosis of FMF and ND, and evaluated the link between the two conditions. FMF-associated NEND was supported by confirmation of both diagnoses and exclusion of other causes of ND.

Results: Eighteen articles were selected. Nine articles reported FMF patients with the following NEND: neutrophilic panniculitis (n = 4), Sweet syndrome (n = 6), and pyoderma gangrenosum (n = 1). None of these cases was supported by histological confirmation, fulfilled diagnostic criteria for definitive or probable FMF, or confirmed the exclusion of all the most frequent diseases associated with NEND. As a result, there is insufficient evidence to support a potential relationship between NEND and FMF.

Conclusions: The association between FMF and NEND remains unclear. In FMF patients with NEND, every differential diagnosis and alternative cause of NEND should be excluded before drawing any conclusions about a potential causal relationship.

目的：家族性地中海热（FMF）是最常见的单基因自身炎症性疾病。它与MEFV突变有关。其主要特征是反复发作的发热和浆膜炎。患者可出现皮肤病学表现，如丹毒样红斑，通常被认为是中性粒细胞性皮肤病 (ND)。有人建议 FMF 可以与其他类型的 ND 相关联。我们的目的是对文献进行系统回顾，以评估 ND 和 FMF 之间的联系。

方法：使用 MEDLINE 对 1946 年至 2018 年的文献进行系统回顾。三位独立研究人员确定了与 FMF 相关的非丹毒样红斑中性粒细胞性皮肤病 (NEND) 的报告，选择了建立 FMF 和 ND 诊断的标准，并评估了这两个条件之间的联系。FMF 相关的 NEND 得到确认诊断和排除 ND 其他原因的支持。

结果：选择了18篇文章。九篇文章报道了 FMF 患者的以下 NEND：中性粒细胞性脂膜炎（n = 4）、Sweet 综合征（n = 6）和坏疽性脓皮病（n = 1）。这些病例都没有得到组织学确认的支持，也没有达到确定或可能的 FMF 的诊断标准，或者证实排除了与 NEND 相关的所有最常见疾病。因此，没有足够的证据支持 NEND 和 FMF 之间的潜在关系。

结论：FMF 和 NEND 之间的关联仍不清楚。在患有 NEND 的 FMF 患者中，在得出有关潜在因果关系的任何结论之前，应排除 NEND 的所有鉴别诊断和替代原因。