Hazan Ezra, Griffin Thomas D, Jabbour Serge A, Keller Matthew S
Department of Dermatology and Cutaneous Biology, Sidney Kimmel Medical College, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania, USA.
Division of Endocrinology, Diabetes and Metabolic Diseases, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania, USA.
Cutis. 2020 Jan;105(1):E6-E10.
Lichen myxedematosus (LM), commonly referred to as papular mucinosis, is a rare papular eruption defined by mucin deposition in the dermis. Scleromyxedema (SM) is a generalized papular and sclerodermoid form of LM. It is a progressive disease of unknown etiology with systemic manifestations that cause serious morbidity and mortality. Current criteria list thyroid dysfunction as an exclusion for the diagnosis of SM. Cases of LM associated with thyroid dysfunction have been defined as atypical. We describe a patient with uncontrolled hypothyroidism due to Hashimoto thyroiditis who subsequently developed a diffuse papular eruption with systemic signs and symptoms attributable to SM. Diagnostic workup, including laboratory studies and histologic specimens from the skin and muscle, were consistent with SM. Furthermore, our patient responded clinically to intravenous immunoglobulin (IVIg) and lenalidomide. We discuss the diagnostic criteria, differential diagnoses, and diagnostic challenges associated with LM in association with thyroid dysfunction. We propose that the presence of thyroid disease should not preclude the diagnosis of SM. Finally, we add to the case reports and series of successful treatments of SM with IVIg and lenalidomide.
黏液水肿性苔藓（LM），通常称为丘疹性黏蛋白病，是一种罕见的丘疹性皮疹，其特征为真皮内有黏蛋白沉积。硬化性黏液水肿（SM）是LM的一种全身性丘疹和硬皮病样形式。它是一种病因不明的进行性疾病，具有全身表现，可导致严重的发病率和死亡率。目前的标准将甲状腺功能障碍列为SM诊断的排除标准。与甲状腺功能障碍相关的LM病例被定义为非典型病例。我们描述了一名因桥本甲状腺炎导致甲状腺功能减退未得到控制的患者，该患者随后出现了弥漫性丘疹性皮疹，并伴有归因于SM的全身症状和体征。包括实验室检查以及皮肤和肌肉组织学标本在内的诊断性检查结果与SM一致。此外，我们的患者对静脉注射免疫球蛋白（IVIg）和来那度胺有临床反应。我们讨论了与甲状腺功能障碍相关的LM的诊断标准、鉴别诊断和诊断挑战。我们提出，甲状腺疾病的存在不应排除SM的诊断。最后，我们补充了关于IVIg和来那度胺成功治疗SM的病例报告和系列报道。
