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原发性皮肤边缘区淋巴瘤伴发继发于脚趾的冻疮样狼疮。

Primary cutaneous marginal zone lymphoma presenting with secondary chilblains of the toes.

机构信息

Internal Medicine Residency, Trinity Health Saint Mary's - Grand Rapids, Grand Rapids, Michigan, USA

Internal Medicine Residency, Trinity Health Saint Mary's - Grand Rapids, Grand Rapids, Michigan, USA.

出版信息

BMJ Case Rep. 2023 Aug 27;16(8):e255894. doi: 10.1136/bcr-2023-255894.

DOI:10.1136/bcr-2023-255894
PMID:37640411
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10462931/
Abstract

Cutaneous lymphoproliferative disorders include cutaneous manifestations of systemic B-cell or T-cell lymphoma and primary cutaneous lymphomas. Primary cutaneous B-cell lymphomas are subcategorised into four groups: primary cutaneous marginal zone lymphoma (PCMZL), primary cutaneous follicle centre lymphoma, primary cutaneous diffuse large B-cell lymphoma, leg-type and EBV-positive mucocutaneous tumour. Each of these cutaneous lymphoproliferative disorders has unique clinical and pathological features necessitating biopsy and staging to establish proper treatment. We present a case of PCMZL manifesting as secondary chilblains of the toes that was diagnosed with punch skin biopsy. The patient's chilblains resolved with rituximab. This case underscores the importance of considering PCMZL and other cutaneous lymphoproliferative disorders in patients with dermatological manifestations and wounds refractory to first-line treatment.

摘要

皮肤淋巴增生性疾病包括系统性 B 细胞或 T 细胞淋巴瘤和原发性皮肤淋巴瘤的皮肤表现。原发性皮肤 B 细胞淋巴瘤分为四组:原发性皮肤边缘区淋巴瘤(PCMZL)、原发性皮肤滤泡中心淋巴瘤、原发性皮肤弥漫性大 B 细胞淋巴瘤、腿型和 EBV 阳性黏膜皮肤肿瘤。这些皮肤淋巴增生性疾病中的每一种都具有独特的临床和病理特征,需要进行活检和分期以确定适当的治疗。我们报告了一例表现为继发于脚趾冻疮的 PCMZL 病例,该病例通过皮肤打孔活检确诊。患者的冻疮在接受利妥昔单抗治疗后消退。该病例强调了在具有皮肤表现和对一线治疗无反应的伤口的患者中,考虑 PCMZL 和其他皮肤淋巴增生性疾病的重要性。

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Epidemiology of Marginal Zone Lymphoma.边缘区淋巴瘤的流行病学
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Arch Dermatol. 2005 Sep;141(9):1139-45. doi: 10.1001/archderm.141.9.1139.
10
Clinical and histopathologic features and immunologic variables in patients with severe chilblains. A study of the relationship to lupus erythematosus.严重冻疮患者的临床、组织病理学特征及免疫变量。与红斑狼疮关系的研究。
Medicine (Baltimore). 2001 May;80(3):180-8. doi: 10.1097/00005792-200105000-00004.