A rare presentation of Rosai-Dorfman-Destombes disease with central nervous system involvement and cutaneous wounds.

IF 1.5 4区医学 Q3 DERMATOLOGY Journal of wound care Pub Date : 2024-05-01 DOI:10.12968/jowc.2024.33.Sup5.S10

Lauren Workman, Lauren Fang, Samar Ayoub, Karen Bach, Richard Simman

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Abstract

Objective: The aim of this case report is to investigate an uncommon presentation of Rosai-Dorfman-Destombes (RDD) disease, and discuss possible differential diagnoses and treatment options for this pathology. RDD is a rare disorder of histiocytes that typically presents in patients as painless cervical lymphadenopathy. However, this case involves a patient with the central nervous system (CNS) type of RDD who later developed cutaneous lesions.

Method: Several differential diagnoses were examined, including hidradenitis suppurativa, pilonidal cyst and pressure ulcers. It is important to be able to exclude these diagnoses based on the presentation, patient demographic and wound location.

Results: Biopsies verified the presence of RDD in the patient's suprasellar hypothalamic mass and skin lesions, confirming the patient had both CNS-RDD and cutaneous-RDD in the absence of lymphadenopathy.

Conclusion: Recognising the unique manifestations of rare diseases such as RDD prevents delay of proper intervention and treatment.

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一种罕见的罗赛-多夫曼-戴斯通贝病，伴有中枢神经系统受累和皮肤伤口。

目的：本病例报告旨在研究罗赛-多夫曼-多斯通布病（RDD）的一种不常见表现，并讨论该病症可能的鉴别诊断和治疗方案。RDD 是一种罕见的组织细胞疾病，患者通常表现为无痛性颈淋巴结病。然而，本病例中一名患者患有中枢神经系统（CNS）型RDD，后来出现了皮肤病变：方法：研究了几种鉴别诊断，包括化脓性扁桃体炎、皮样囊肿和压疮。根据患者的表现、人口统计学特征和伤口位置排除这些诊断非常重要：活组织检查证实，患者下丘脑上部肿块和皮肤病变中存在 RDD，证实患者同时患有中枢神经系统 RDD 和皮肤 RDD，但没有淋巴结病：结论：认识 RDD 等罕见疾病的独特表现可避免延误适当的干预和治疗。

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来源期刊

Journal of wound care DERMATOLOGY-

CiteScore

2.90

自引率

10.50%

发文量

215

期刊介绍： Journal of Wound Care (JWC) is the definitive wound-care journal and the leading source of up-to-date research and clinical information on everything related to tissue viability. The journal was first launched in 1992 and aimed at catering to the needs of the multidisciplinary team. Published monthly, the journal’s international audience includes nurses, doctors and researchers specialising in wound management and tissue viability, as well as generalists wishing to enhance their practice. In addition to cutting edge and state-of-the-art research and practice articles, JWC also covers topics related to wound-care management, education and novel therapies, as well as JWC cases supplements, a supplement dedicated solely to case reports and case series in wound care. All articles are rigorously peer-reviewed by a panel of international experts, comprised of clinicians, nurses and researchers. Specifically, JWC publishes: High quality evidence on all aspects of wound care, including leg ulcers, pressure ulcers, the diabetic foot, burns, surgical wounds, wound infection and more The latest developments and innovations in wound care through both preclinical and preliminary clinical trials of potential new treatments worldwide In-depth prospective studies of new treatment applications, as well as high-level research evidence on existing treatments Clinical case studies providing information on how to deal with complex wounds Comprehensive literature reviews on current concepts and practice, including cost-effectiveness Updates on the activities of wound care societies around the world.