NYU Multiple Sclerosis Comprehensive Care Center, Department of Neurology, New York University School of Medicine, New York, NY, USA.
Department of Pathology and Laboratory Medicine, Weill Cornell Medicine, New York, NY, USA.
Curr Neurol Neurosci Rep. 2019 Feb 12;19(3):11. doi: 10.1007/s11910-019-0929-8.
To describe diverse neurologic and neuroradiologic presentations of two rare, immunologically mediated skin conditions: Sweet disease and localized scleroderma (morphea).
Core syndromes of neuro-Sweet disease (NSD) are steroid responsiveness, recurrent meningitis, and encephalitis. Focal neurologic, neuro-vascular, and neuro-ophthalmologic syndromes have been reported recently in NSD. A variety of steroid-sparing treatments and biologics have been used for relapsing NSD. Localized craniofacial scleroderma is associated with seizures, headaches, and, less commonly, focal deficits and cognitive decline. Immunosuppressive therapy may be required in patients with disease progression; some refractory cases have responded to IL-6 inhibition. Our review provides an up-to-date reference for neurologists faced with a patient with a history or skin findings consistent with Sweet disease or localized scleroderma. We hope that it will stimulate collaborative studies aimed at unraveling the pathogenesis of these disorders, better characterization of their neurologic manifestations, and discovery of optimal therapeutic solutions.
描述两种罕见的免疫介导性皮肤疾病——Sweet 病和局限性硬皮病（硬斑病）的多种神经和神经放射学表现。
神经Sweet 病（neuro-Sweet disease，NSD）的核心综合征是类固醇反应性、复发性脑膜炎和脑炎。最近报道了 NSD 的局灶性神经、神经血管和神经眼科综合征。对于复发性 NSD，已使用多种类固醇保留治疗和生物制剂。局限性颅面硬皮病与癫痫、头痛有关，较少见的还有局灶性缺损和认知功能下降。有疾病进展的患者可能需要免疫抑制治疗；一些难治性病例对白细胞介素-6 抑制有反应。我们的综述为神经科医生提供了一个最新的参考，他们可能会遇到符合 Sweet 病或局限性硬皮病病史或皮肤表现的患者。我们希望它将激发旨在阐明这些疾病发病机制、更好地描述其神经表现以及发现最佳治疗方案的合作研究。
