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罗萨伊-多夫曼病病例报告

Rosai-Dorfman Disease Case Report.

作者信息

Altamimi Naif M, Li Vivian, Kahn Hershel, Shaikh Gibran

机构信息

Department of Dermatology, University of Hail, Hail, Saudi Arabia.

Lake Erie College of Osteopathic Medicine, Erie, PA, USA.

出版信息

Case Rep Dermatol. 2025 May 9;17(1):299-303. doi: 10.1159/000546278. eCollection 2025 Jan-Dec.

DOI:10.1159/000546278
PMID:40671735
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12266700/
Abstract

INTRODUCTION

Rosai-Dorfman disease (RDD) is a rare histiocytic disorder that is characterized by the accumulation of histiocytes in various tissues, most commonly the lymph nodes. Cutaneous RDD is a rare presentation, accounting for less than 10% of all RDD cases.

CASE PRESENTATION

An 18-year-old female presented for the first time with a dome-like lesion over an indurated plaque. Both lesions were biopsied, and findings suggested RDD. The patient was referred to an oncologist with a subsequent positron emission tomography scan showing hypermetabolic subcutaneous uptake on bilateral thighs and bilateral lumbar lesions, likely corresponding to the patient's biopsy-proven histiocytosis in addition to a diffuse increased tracer uptake in the thoracic aorta and atria.

CONCLUSION

A high degree of suspicion is necessary when dermatologists notice two different morphologies present in proximity to rule out a single pathological process. This case is unique because of the rarity of the condition as well as the clinical presentation of two different morphologies of an indurated plaque and a dome-like lesion on the same area of the body.

摘要

引言

罗萨伊-多夫曼病(RDD)是一种罕见的组织细胞疾病,其特征是组织细胞在各种组织中积聚,最常见于淋巴结。皮肤型RDD是一种罕见的表现形式,占所有RDD病例的不到10%。

病例介绍

一名18岁女性首次就诊时,在一个硬结斑块上出现一个圆顶状病变。对这两个病变均进行了活检,结果提示为RDD。该患者被转诊至肿瘤学家处,随后的正电子发射断层扫描显示双侧大腿和双侧腰部病变处有高代谢的皮下摄取,除了胸主动脉和心房有弥漫性示踪剂摄取增加外,这可能与患者经活检证实的组织细胞增多症相对应。

结论

当皮肤科医生注意到相邻部位出现两种不同形态时,必须高度怀疑以排除单一病理过程。该病例独特之处在于这种疾病的罕见性以及在身体同一部位出现硬结斑块和圆顶状病变这两种不同形态的临床表现。

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本文引用的文献

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Intravenous immunoglobulin in the management of refractory lupus profundus.静脉注射免疫球蛋白治疗狼疮性深部组织病。
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Cutaneous Rosai-Dorfman Disease: A Treatment Challenge.皮肤型罗萨伊-多夫曼病:一项治疗挑战。
Dermatol Ther (Heidelb). 2021 Aug;11(4):1443-1448. doi: 10.1007/s13555-021-00557-1. Epub 2021 Jun 18.
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Rosai-Dorfman Disease Displays a Unique Monocyte-Macrophage Phenotype Characterized by Expression of OCT2.罗萨伊-多夫曼病表现出一种独特的单核细胞-巨噬细胞表型,其特征为OCT2的表达。
Am J Surg Pathol. 2021 Jan;45(1):35-44. doi: 10.1097/PAS.0000000000001617.
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Primary cutaneous Rosai-Dorfman disease; a case-based review of a diagnostically and therapeutically challenging rare variant.原发性皮肤Rosai-Dorfman病;基于病例对一种诊断和治疗具有挑战性的罕见变体的综述
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Cutaneous Rosai-Dorfman Disease of the Face: A Comprehensive Literature Review and Case Report.面部皮肤型罗萨伊-多夫曼病:一项全面的文献综述及病例报告
J Oral Maxillofac Surg. 2016 Mar;74(3):528-40. doi: 10.1016/j.joms.2015.09.017. Epub 2015 Sep 25.
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Rosai-Dorfman disease: tumor biology, clinical features, pathology, and treatment.罗萨伊-多夫曼病:肿瘤生物学、临床特征、病理学及治疗
Cancer Control. 2014 Oct;21(4):322-7. doi: 10.1177/107327481402100408.
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