Elwyn Lacey B, Combs Angela, Villada Gabriel, Poulos Evangelos, Cespedes Yvana, Rodriguez-Vazquez Georgette, Albitar Maher, Karai Laszlo
Larking Community Hospital, Miami, FL.
Global Pathology Laboratory, Aurora Diagnostics, Miami Lakes, FL.
Am J Dermatopathol. 2019 Jul;41(7):505-510. doi: 10.1097/DAD.0000000000001335.
Lichen myxedematosus is a chronic cutaneous mucinosis that can present on a spectrum from localized cutaneous lesions to systemic disease of scleromyxedema. The clinical presentation of localized cutaneous lichen myxedematosus is waxy lichenoid papules, nodules, and/or plaques that have histopathologic findings of mucin deposition and a variable degree of fibroblast proliferation. There is an absence of serum paraproteins, and there are no other systemic causes of cutaneous mucinosis such as thyroid disease. The pathogenesis of lichen myxedematosus is unknown. We report 3 cases of localized cutaneous lichen myxedematosus with a light chain-restricted plasmacytic component by in situ hybridization. Our findings deliver an insight for disease pathogenesis and highlight for the first time, the significance of plasma cells in lesions of localized cutaneous lichen myxedematosus. We suggest that plasma cell light chain restriction could represent a clue to distinguish localized cutaneous disease from systemic disease.
黏液水肿性苔藓是一种慢性皮肤黏蛋白沉积症，其表现范围从局限性皮肤病变到硬肿病的系统性疾病。局限性皮肤黏液水肿性苔藓的临床表现为蜡样苔藓样丘疹、结节和/或斑块，其组织病理学表现为黏蛋白沉积和成纤维细胞不同程度的增殖。血清中无副蛋白，且不存在其他导致皮肤黏蛋白沉积症的系统性病因，如甲状腺疾病。黏液水肿性苔藓的发病机制尚不清楚。我们报告了3例局限性皮肤黏液水肿性苔藓病例，通过原位杂交发现其存在轻链受限的浆细胞成分。我们的研究结果为疾病发病机制提供了见解，并首次强调了浆细胞在局限性皮肤黏液水肿性苔藓病变中的重要性。我们认为浆细胞轻链受限可能是区分局限性皮肤疾病和系统性疾病 的一个线索。
