每日一题_2023.05.05

A 66-year-old man presented with an 8-month history of painful skin tightening and swelling of his arms and legs. The symptoms had progressed on glucocorticoids and methotrexate. On physical exam, there was symmetric, woody induration of the trunk, arms, and legs, sparing the hands and feet. The involved skin had a dimpled appearance. Elevation of the arms resulted in visible indentions along the course of superficial veins. Elbow contractures were also noted. What is the most likely diagnosis?

患者男，66岁，因四肢皮肤紧绷、肿胀伴疼痛8个月就诊。糖皮质激素和甲氨蝶呤治疗后症状进展。体格检查发现躯干、手臂和腿部对称性木质硬结，手和脚未见硬结。受累皮肤呈酒窝状。手臂的抬高导致了沿浅静脉走行可见的凹痕。肘关节挛缩也被记录。最有可能的诊断是什么?

A. Eosinophilic fasciitis 嗜酸性筋膜炎

B. Myxedema 粘液水肿

C. Scleredema 硬化病

D. Scleroderma 硬皮病

E. Scleromyxedema 硬化性粘液水肿

答案解析：

沿浅静脉走行可见的凹陷与“沟槽征”一致，这是在嗜酸性筋膜炎中看到的一种表现。值得注意的是实验室检查显示的绝对嗜酸性粒细胞增多。行左臂皮肤活检，组织病理学检查示真皮深层网状层及皮下血管周围及间质淋巴细胞、嗜酸性粒细胞炎性浸润。给予静脉注射甲泼尼龙、口服吗替麦考酚酯、静脉注射丙种球蛋白治疗。

正确答案：A