Li Yan, Wang Hui, Wang Aiqin, Zhao Guowei
Department of Dermatology, ZiBo Central Hospital, Zibo, China.
J Dermatol. 2024 Jan;51(1):140-144. doi: 10.1111/1346-8138.16994. Epub 2023 Oct 13.
Scleromyxedema (SMX) is a rare disease of unknown cause. It is a chronic, progressive, metabolic disorder characterized by a generalized papular and scleroderma-like rash, as well as a subtype of lichen myxedematosus. Dermato-neuro syndrome (DNS) is a rare neurological complication of SMX. It has flu-like prodromal symptoms; consists of a triad of fever, coma, and seizures; and can be life-threatening. We describe a patient with SMX complicated by DNS after infection with COVID-19. Her symptoms resolved after treatment with acyclovir and low-dose glucocorticoids, suggesting that DNS seizures may have a viral cause. Her skin lesions also improved after seven courses of intravenous immunoglobulin treatment, confirming that intravenous immunoglobulin is effective in these cases.
硬化性黏液水肿（SMX）是一种病因不明的罕见疾病。它是一种慢性、进行性代谢紊乱疾病，其特征为全身性丘疹和硬皮病样皮疹，也是黏液水肿性苔藓的一种亚型。皮肤神经综合征（DNS）是SMX一种罕见的神经并发症。它有类似流感的前驱症状；由发热、昏迷和癫痫发作三联征组成，可能危及生命。我们描述了一名感染新冠病毒后并发DNS的SMX患者。经阿昔洛韦和小剂量糖皮质激素治疗后，她的症状得到缓解，这表明DNS癫痫发作可能由病毒引起。经过七个疗程的静脉注射免疫球蛋白治疗后，她的皮肤病变也有所改善，证实静脉注射免疫球蛋白对这些病例有效。
