苔藓粘液症(LM)是一种慢性皮肤粘液病,可表现为局部皮肤病变或全身性全身性疾病,称为巩膜水肿。鉴别诊断取决于临床表现的组合,血清学研究,和组织病理学检查。目前,尚未阐明将局部LM与巩膜水肿区分开来的公认和公认的组织病理学特征。我们最近的出版物,以及回顾性文献综述,提示轻链限制性浆细胞群的存在代表了诊断局部LM的独特组织病理学线索。在这份报告中,我们提供了另外两个带有λ轻链限制性浆细胞的局部LM病例,以及与我们以前的出版物相似的临床和组织病理学发现。这些病例支持我们的理论,即轻链限制的浆细胞微环境主要归因于局部LM的发病机理。因此,我们认为这些病例构成了局部LM的临床和病理新变体,并将其命名为具有轻链限制性浆细胞的原发性局部皮肤LM。
Lichen myxedematosus (LM) is a chronic cutaneous mucinosis that can present as a localized skin lesion or as a generalized systemic disease termed scleromyxedema. The differential diagnosis is determined by a combination of clinical presentation, serological studies, and histopathological examination. Currently, well-established and accepted histopathological features to distinguish localized LM from scleromyxedema have not been elucidated. Our recent publication, together with a retrospective literature review, suggests that the presence of groups of light chain-restricted plasma cells represents a distinct histopathological clue for the diagnosis of localized LM. In this report, we provide two additional cases of localized LM with lambda light chain-restricted plasma cells, together with clinical and histopathological findings that are similar to our previous publication. These cases support our theory that the light chain-restricted plasmacytic microenvironment is primarily attributed to the pathogenesis of localized LM. Therefore, we consider these cases to constitute a clinically and pathologically new variant of localized LM and name it primary localized cutaneous LM with light chain-restricted plasma cells.