Department of Dermatology, University of British Columbia, Vancouver, BC, Canada.
J Cutan Med Surg. 2013 Jan-Feb;17(1):69-73. doi: 10.2310/7750.2012.11125.
Lichen myxedematosus (LM) is a rare idiopathic disorder characterized by papules, plaques, and/or nodules in the skin secondary to mucin deposition and variable dermal fibrosis in the absence of thyroid disease. Case reports are the predominant literature on LM due to its rarity, and the data are limited regarding the disease. No standard treatment regimen exists, and the response to treatment varies.
This report adds to the limited literature on atypical LM associated with thyroid dysfunction and contributes to the growing body of evidence supporting intravenous immunoglobulin (IVIg) as effective therapy for extensive cases.
We report an unusual case of atypical LM associated with hypothyroidism, central nervous system (CNS) disturbances, and atrial fibrillation in a 64-year-old male. The patient experienced remarkable improvement within 3 months of beginning IVIg treatment; however, he required repeat therapy due to the recurrence of CNS symptoms 3 months after treatment.
We agree with previous authors that thyroid dysfunction alone should not preclude a diagnosis of LM. Additionally, we contribute to the increasing evidence of IVIg being an effective treatment, particularly in the setting of systemic complications or acute worsening of LM.
苔藓样黏蛋白病（lichen myxedematosus，LM）是一种罕见的特发性疾病，其特征为皮肤出现丘疹、斑块和/或结节，这是由于黏蛋白沉积和可变的皮肤纤维化所致，而甲状腺疾病并不常见。由于其罕见性，病例报告是关于 LM 的主要文献，关于该疾病的数据有限。由于没有标准的治疗方案，因此治疗反应也各不相同。
本报告补充了与甲状腺功能障碍相关的非典型 LM 的有限文献，并为越来越多的证据支持静脉注射免疫球蛋白（intravenous immunoglobulin，IVIg）作为广泛病例的有效治疗方法提供了依据。
我们报告了一例罕见的非典型 LM 病例，该病例与甲状腺功能减退、中枢神经系统（central nervous system，CNS）紊乱和心房颤动相关，发生于一名 64 岁男性。该患者在开始 IVIg 治疗后 3 个月内得到了显著改善；然而，由于治疗后 3 个月 CNS 症状再次出现，他需要重复治疗。
我们同意之前的作者的观点，即单纯的甲状腺功能障碍不应排除 LM 的诊断。此外，我们为 IVIg 作为一种有效治疗方法的证据不断增加做出了贡献，特别是在存在系统性并发症或 LM 急性恶化的情况下。
