Department of Dermatology, Shenzhen University General Hospital, Shenzhen, Shenzhen, China.
Longhua District People's Hospital, Shenzhen, China.
Front Immunol. 2024 Oct 24;15:1492547. doi: 10.3389/fimmu.2024.1492547. eCollection 2024.
To date, the pathogenesis of Kimura's disease remains unclear, there is no unified diagnostic criterion, the clinical phenotype shows considerable heterogeneity, and there is a lack of optimal treatment strategies. Due to its rarity, treatment strategies for KD are still under exploration. This paper reports a case of a 37-year-old Chinese female presenting with generalized erythematous papules and pruritic eruptions for 12 years, followed by the onset of limb swellings 3 years later, ultimately diagnosed as Kimura's disease. Considering the patient's multiple lymphadenopathies and limb swellings with concurrent atopic dermatitis, the treatment regimen included initial dupilumab dosage of 600 mg (300 mg administered in two injections), followed by subcutaneous injections of 300 mg every two weeks for four months. Concurrent oral corticosteroid therapy (methylprednisolone, initial dose 16 mg/kg/day, gradually tapered with tumor regression) was also administered. Following treatment, the patient did not experience severe adverse effects, and the multiple nodules markedly decreased in size. Additionally, serum IgE levels, eosinophil, and basophil counts showed significant reductions. These results demonstrate the significant efficacy of dupilumab combined with oral corticosteroids in treating Kimura's disease with concurrent atopic dermatitis.
迄今为止，木村病的发病机制仍不清楚，尚无统一的诊断标准，临床表型表现出相当大的异质性，并且缺乏最佳的治疗策略。由于其罕见性，KD 的治疗策略仍在探索中。本文报告了一例 37 岁的中国女性，她因全身性红斑丘疹和瘙痒性皮疹 12 年，随后出现四肢肿胀 3 年后，最终被诊断为木村病。鉴于患者存在多处淋巴结病和四肢肿胀伴特应性皮炎，治疗方案包括初始使用度普利尤单抗 600mg（分两次注射，每次 300mg），随后每两周皮下注射 300mg，共 4 个月。同时给予口服皮质类固醇治疗（甲泼尼龙，初始剂量 16mg/kg/天，随着肿瘤缩小逐渐减量）。治疗后，患者未出现严重不良反应，多发结节明显缩小。此外，血清 IgE 水平、嗜酸性粒细胞和嗜碱性粒细胞计数均显著降低。这些结果表明，度普利尤单抗联合口服皮质类固醇治疗并发特应性皮炎的木村病具有显著疗效。
