Rheumatology Department, Universidad Autónoma de Nuevo León, Facultad de Medicina y Hospital Universitario "Dr. José Eleuterio González", Monterrey, Mexico.
Dermatology Department, Universidad Autónoma de Nuevo León, Facultad de Medicina y Hospital Universitario "Dr. José Eleuterio González", Monterrey, Mexico.
J Dermatolog Treat. 2022 May;33(3):1765-1768. doi: 10.1080/09546634.2021.1890682. Epub 2021 Feb 21.
Eosinophilic fasciitis (EF) is an uncommon autoimmune connective tissue disorder characterized by edema, erythema, and subsequent induration of the extremities. It is commonly treated with corticosteroids but there is no treatment ladder for immunosuppressants or steroid-sparing agents. We report two EF cases treated effectively with mycophenolate mofetil (MMF) or mycophenolic acid (MPA) and present a literature review. We performed a MEDLINE search using the keywords 'eosinophilic fasciitis', 'Shulman syndrome', 'mycophenolic acid', or 'mofetil mycophenolate', and found 8 articles with 27 cases in which MMF or MPA was used. Twenty-nine cases were reviewed (2 reported herein and 27 from the literature search); all patients received a combination of systemic corticosteroids and MMF. MMF/MPA were given as a steroid-sparing agent in 27 (93.1%), in 1 (3.4%) as adjunctive therapy with other immunosuppressants, and in one, as monotherapy 1 (3.4%). Nineteen had a complete response, 6, a partial response, and 2 were unresponsive to diverse immunomodulators; in 2 cases, the outcome was not reported. MMF and MPA show promising therapeutic results and could be a treatment option to reduce corticosteroid related side effects.
嗜酸性筋膜炎（EF）是一种罕见的自身免疫性结缔组织疾病，其特征为四肢水肿、红斑，随后出现硬结。该病通常采用皮质类固醇治疗，但免疫抑制剂或皮质类固醇节约剂并无治疗阶梯。我们报告了两例嗜酸性筋膜炎病例，采用麦考酚酸酯（MPA）或麦考酚酯（MMF）治疗效果显著，并进行了文献复习。我们使用关键词“嗜酸性筋膜炎”、“舒尔曼综合征”、“麦考酚酸”或“霉酚酸酯”进行了 MEDLINE 检索，共找到 8 篇文章，其中有 27 例使用 MMF 或 MPA。共回顾了 29 例病例（本文报告 2 例，文献检索 27 例）；所有患者均接受了全身性皮质类固醇和 MMF 的联合治疗。27 例（93.1%）将 MMF/MPA 作为皮质类固醇节约剂，1 例（3.4%）作为其他免疫抑制剂的辅助治疗，1 例（3.4%）作为单药治疗。19 例患者完全缓解，6 例部分缓解，2 例对多种免疫调节剂无反应；2 例病例的结果未报道。MMF 和 MPA 显示出有前景的治疗效果，可能是减少皮质类固醇相关副作用的一种治疗选择。
