Tang Ping, Chen Jing-Si, Wang Hua, Yang Huan
Department of Dermatology, Ministry of Education Key Laboratory of Child Development and Disorders, National Clinical Research Center for Child Health and Disorders, Children's Hospital of Chongqing Medical University, Chongqing, China.
China International Science and Technology Cooperation base of Child development and Critical Disorders, Chongqing, China.
Case Rep Dermatol. 2022 Jun 27;14(2):169-177. doi: 10.1159/000525008. eCollection 2022 May-Aug.
The characteristics and treatments of febrile ulceronecrotic Mucha-Habermann disease (FUMHD) are not well-understood. We reported a FUMHD case, and searched Medline, Embase, Pubmed, Scopus, and Web of Science from inception to June 16, 2021, to perform a systematic review to synthesize its characteristics and treatments. Seventy-eight reports, including 84 people were eligible. Most of them were male (62/83, 74.7%), with high fever state (50/80, 62.5% had a high fever of 39°C or above), and with more positive skin bacterial cultures (31/41, 75.6%). Adults were associated with a higher risk of death (OR = 12.976, 95% CI: 1.049, 160.504, = 0.046), but not positive blood bacterial cultures ( = 0.102). Systematic corticosteroids combination with other immunosuppressants (methotrexate or cyclosporine) were associated with significantly more effective cases (26/31 = 83.9%, χ = 4.065, = 0.044). Furthermore, no significant differences between the low-dose and high-dose systematic corticosteroid groups were detected in treatment validation ( > 0.05). Overall, FUMHD was associated with male patients, high fever, and positive skin bacterial cultures. Early combination therapy with lower doses of corticosteroids and methotrexate or cyclosporine may be an optimal choice for the treatment of FUMHD.
发热性溃疡性坏死性穆查-哈伯曼病（FUMHD）的特征及治疗方法尚未完全明确。我们报告了1例FUMHD病例，并检索了从建库至2021年6月16日的Medline、Embase、Pubmed、Scopus和科学网，以进行系统综述，综合其特征及治疗方法。纳入78篇报告，共84例患者。其中男性居多（62/83，74.7%），多处于高热状态（50/80，62.5%体温达到或超过39℃），皮肤细菌培养阳性率较高（31/41，75.6%）。成人死亡风险较高（OR = 12.976，95%CI：1.049，160.504， = 0.046），但血细菌培养阳性情况无差异（ = 0.102）。系统性使用糖皮质激素联合其他免疫抑制剂（甲氨蝶呤或环孢素）的有效病例显著更多（26/31 = 83.9%，χ = 4.065， = 0.044）。此外，低剂量和高剂量系统性糖皮质激素组在治疗有效性方面未检测到显著差异（ > 0.05）。总体而言，FUMHD与男性患者、高热及皮肤细菌培养阳性相关。早期联合使用低剂量糖皮质激素与甲氨蝶呤或环孢素可能是治疗FUMHD的最佳选择。
