Marcelus Christina, Jimenez Amber, Zussman Jamie, Hansen Christopher B, Sontheimer Richard D
Department of Dermatology, Spencer Fox Eccles School of Medicine, University of Utah, Salt Lake City, Utah, USA.
Dermatol Online J. 2022 Oct 15;28(5). doi: 10.5070/D328559245.
Morphea presenting clinically with nodular or keloidal skin changes is extremely rare. Nodular scleroderma or keloidal morphea presenting in a linear distribution is even more uncommon. We present an otherwise healthy young woman with unilateral, linear, nodular scleroderma and review the somewhat confounding earlier literature in this area. To date, this young woman's skin changes have proven refractory to oral hydroxychloroquine and ultraviolet A1 phototherapy. Several aspects of this case including the patient's family history of Raynaud disease, her nodular sclerodermatous skin lesions, and the presence of U1RNP autoantibodies raised concern about her management with respect to future risk of developing systemic sclerosis.
临床上表现为结节状或瘢痕疙瘩样皮肤改变的硬斑病极为罕见。呈线性分布的结节性硬皮病或瘢痕疙瘩样硬斑病则更为少见。我们报告一例身体健康的年轻女性，患有单侧线性结节性硬皮病，并回顾该领域早期有些令人困惑的文献。迄今为止，该年轻女性的皮肤改变对口服羟氯喹和紫外线A1光疗均无反应。该病例的几个方面，包括患者雷诺病的家族史、结节性硬皮病皮肤损害以及U1RNP自身抗体的存在，引发了对其未来发生系统性硬化症风险的管理担忧。
