Internal Medicine Department, Hospital Costa del Sol, Marbella, Spain.
Dermatology Department, Hospital Costa del Sol, Marbella, Spain.
Rheumatol Int. 2021 Aug;41(8):1531-1539. doi: 10.1007/s00296-021-04787-6. Epub 2021 Jan 23.
Eosinophilic fasciitis (EF) is characterized by symmetrical thickening of subcutaneous muscular fascia, causing skin induration with wrinkles and prominent hair follicles: the classic peau d'orange. Eosinophilia is a characteristic-albeit not universal-finding. We present the case of a 43-year-old pregnant woman diagnosed with EF during pregnancy who had extensive cutaneous involvement and severe functional repercussions, including worsening of lung function and intrauterine growth restriction as a possible complication. Treatment with prednisone was initiated during gestation and it was necessary to increase the dose. After delivery, methotrexate treatment was initiated and the corticosteroid dose progressively decreased, with progressive worsening in the torso and abdomen and secondary dyspnea due to thoracic pressure. Treatment with infliximab was then initiated, with favorable progress, though residual ankle and tarsal joint stiffness and significant muscular atrophy in the limbs continued. The triggering factor of EF was not identified. In a systematic search of the medical literature, three cases of EF in pregnant woman without clear triggers were found. Interestingly, all three cases progressed favorably with steroid treatment. Apart from this case, there are only seven published cases of infliximab use in the literature, all with moderate or complete response. Infliximab could be an option for corticosteroid-dependent EF with no response to other options.
嗜酸性筋膜炎(EF)的特征为对称性的皮下肌肉筋膜增厚,导致皮肤硬化伴有皱纹和突出的毛囊:典型的橘皮样外观。嗜酸性粒细胞增多是一种特征性表现,但并非普遍存在。我们报告了一例 43 岁的孕妇,在怀孕期间被诊断为 EF,其皮肤广泛受累,并伴有严重的功能影响,包括肺功能恶化和宫内生长受限可能是其并发症。在妊娠期间开始使用泼尼松龙进行治疗,并且需要增加剂量。分娩后,开始使用甲氨蝶呤治疗,皮质类固醇剂量逐渐减少,胸壁压力导致躯干和腹部进一步恶化,并出现继发性呼吸困难。随后开始使用英夫利昔单抗治疗,病情有所好转,但四肢仍有踝关节和跗关节僵硬和明显的肌肉萎缩。EF 的触发因素未被确定。在对医学文献的系统搜索中,发现了 3 例无明确诱因的孕妇 EF 病例。有趣的是,所有 3 例病例均经类固醇治疗后病情好转。除了这例病例,文献中仅有 7 例使用英夫利昔单抗的病例报道,所有病例均有中度或完全缓解。对于对其他治疗方案无反应的依赖于皮质类固醇的 EF,英夫利昔单抗可能是一种选择。