Department of Rheumatology, Alder Hey Children's NHS Foundation Trust, Eaton Road, Liverpool, UK; Department of Women's and Children's Health, Institute of Life Course and Medical Sciences, University of Liverpool, Liverpool, UK.
Division of Pediatric Rheumatology, UPMC Children's Hospital of Pittsburgh, Pittsburgh, USA; Department of Pediatrics, University of Pittsburgh School of Medicine, University of Pittsburgh Scleroderma Center, 4401 Penn Ave Pittsburgh, Pennsylvania, USA, 15224.
Best Pract Res Clin Rheumatol. 2024 Sep;38(3):101987. doi: 10.1016/j.berh.2024.101987. Epub 2024 Sep 17.
Juvenile localized scleroderma is characterised by inflammation which drives fibrosis in skin and soft tissues. The more severe subtypes of localized scleroderma such as linear and craniofacial are more common in children. Additionally, extracutaneous involvement is seen in half of all children and is associated with poorer treatment outcomes and health-related quality of life. Evidence for the management of craniofacial and extracutaneous involvement is lacking and therefore poses a challenge to clinicians. This review aims to provide a practical approach to management of these most challenging features of juvenile localized scleroderma through case studies where we present the available evidence, current recommendations and considerations for management.
局限性幼年型硬皮病的特征为炎症驱动皮肤和软组织纤维化。更严重的局限性硬皮病亚型,如线状和颅面型,在儿童中更为常见。此外,半数儿童有皮肤外受累,与治疗结局较差和健康相关生活质量下降相关。颅面型和皮肤外受累的管理证据不足,因此对临床医生构成挑战。本综述旨在通过病例研究,提供一种实用的方法来管理局限性幼年型硬皮病的这些最具挑战性的特征,我们在病例研究中介绍了现有证据、当前推荐意见和管理注意事项。