意义不明的单克隆性腺病引起的弥漫性平面黄瘤病。

IF 2.9 3区 医学 Q2 DERMATOLOGY Journal of Dermatology Pub Date : 2024-08-21 DOI:10.1111/1346-8138.17432
Marisa Lenga, Jennifer Nam Choi
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引用次数: 0

摘要

一名 60 多岁的妇女因 6 年来逐渐累积的无症状黄色斑块就诊。她的病史包括 6 年的 IgG lambda 轻链亚型意义未定单克隆丙种球蛋白病(MGUS)和高胆固醇血症。体格检查时,她的眼睑、上胸部、上臂前部、腋窝和眶前窝出现了界限清楚的黄色薄斑,但没有粘膜病变(图 1)。组织病理学检查显示,真皮浅层和中层散在聚集着泡沫组织细胞,血管周围有轻度淋巴组织细胞浸润。血脂检查结果显示,低密度脂蛋白胆固醇轻度升高,为122毫克/分升(参考值为100毫克/分升)。患者被诊断为弥漫性平面黄瘤病,伴有 MGUS。由于患者当时不需要治疗多发性肌肉萎缩症,我们与患者讨论了皮肤定向治疗方案,她选择用冷冻疗法治疗左前臂。弥漫性平面黄疽的特点是富含胆固醇的物质在皮肤中分散堆积,家族性高脂血症患者或血脂正常但轻度升高且无家族史的患者均可发病、2 在血脂正常的患者中,有一种在血液病或淋巴增生性疾病的情况下形成弥漫性黄瘤的现象。1 在MGUS(最常见的是IgG kappa亚型)的情况下,黄瘤是通过单克隆IgG和低密度脂蛋白复合物的形成以及随后巨噬细胞对这些复合物的吞噬作用而形成的。本病表现为无症状、对称性、黄色至橙色斑块,最常见于眼睑、颈部、躯干上部和褶皱处。4 组织病理学检查显示真皮中存在弥漫性泡沫细胞,并伴有数量不等的巨细胞、淋巴细胞和泡沫组织细胞。黄疽可自发消退,也可在治疗MGUS后消退,或通过切除、化学磨削、激光烧蚀和冷冻治疗等干预手段消退。3, 4本病例的检查结果具有代表性,可提高临床对MGUS情况下弥漫性黄疽的识别能力。
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Diffuse planar xanthomatosis in the setting of monoclonal gammopathy of undetermined significance

A woman in her 60s presented to the clinic for progressively accumulating, asymptomatic, yellow plaques for 6 years. Her medical history included monoclonal gammopathy of undetermined significance (MGUS) of IgG lambda light chain subtype for 6 years and hypercholesteremia. On physical examination, well-defined, thin, yellow plaques were located on the eyelids, upper chest, anterior upper arms, axillae, and antecubital fossae without mucosal lesions (Figure 1). Histopathological examination revealed scattered collections of foamy histiocytes in the superficial and midreticular dermis with mild perivascular lymphohistiocytic infiltrate. Lipid panel findings demonstrated a mildly elevated low-density lipoprotein cholesterol at 122 mg/dL (reference < 100 mg/dL). The patient was diagnosed with diffuse planar xanthomatosis in the setting of MGUS. Because the patient did not require treatment for MGUS at the time, skin-directed treatment options were reviewed with the patient, and she elected to treat the left anterior arm with cryotherapy. She was lost to follow-up.

Diffuse planar xanthomatosis is characterized by the dispersed accumulation of cholesterol-rich material in the skin and can develop in patients either with familial hyperlipidemia or with normal and slightly elevated lipids without family history.1, 2 In normolipidemic patients, there is a described phenomenon of diffuse xanthomas forming in the setting of hematological or lymphoproliferative disorders.1 Xanthomas form in the setting of MGUS (most commonly the IgG kappa subtype) through the creation of monoclonal IgG and low-density lipoprotein complexes and the subsequent phagocytosis of these complexes by macrophages.3 This disease presents as asymptomatic, symmetric, yellow to orange plaques most commonly on the eyelids, neck, upper trunk, and flexural folds.4 Histopathologic examination demonstrates diffuse foamy cells in the dermis with variable numbers of giant cells, lymphocytes, and foamy histiocytes.4 Xanthomatosis may resolve spontaneously, with the treatment of MGUS, or with intervention like excision, chemabrasion, ablative laser, and cryotherapy.3, 4

This case serves as representative examination findings to improve clinical recognition of diffuse xanthomatosis in the setting of MGUS.

The authors declare no conflicts of interest for this article.

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来源期刊
Journal of Dermatology
Journal of Dermatology 医学-皮肤病学
CiteScore
4.60
自引率
9.70%
发文量
368
审稿时长
4-8 weeks
期刊介绍: The Journal of Dermatology is the official peer-reviewed publication of the Japanese Dermatological Association and the Asian Dermatological Association. The journal aims to provide a forum for the exchange of information about new and significant research in dermatology and to promote the discipline of dermatology in Japan and throughout the world. Research articles are supplemented by reviews, theoretical articles, special features, commentaries, book reviews and proceedings of workshops and conferences. Preliminary or short reports and letters to the editor of two printed pages or less will be published as soon as possible. Papers in all fields of dermatology will be considered.
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