University of Toledo, College of Medicine and Life Science, Toledo, Ohio, US.
Jobst Vascular Institute, ProMedica Health Network, Wound Care Program, Toledo, Ohio, US.
J Wound Care. 2024 May 1;33(Sup5):S10-S13. doi: 10.12968/jowc.2024.33.Sup5.S10.
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.
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.
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.
Recognising the unique manifestations of rare diseases such as RDD prevents delay of proper intervention and treatment.
本病例报告旨在研究罗萨达-多夫曼-德斯东贝(RDD)病的一种不常见表现,并讨论该病理学的可能鉴别诊断和治疗选择。RDD 是一种罕见的组织细胞疾病,通常在患者中表现为无痛性颈淋巴结病。然而,本病例涉及中枢神经系统(CNS)型 RDD 患者,随后出现皮肤病变。
检查了几种鉴别诊断,包括化脓性汗腺炎、藏毛囊肿和压疮。根据表现、患者人口统计学和伤口位置,排除这些诊断很重要。
活检证实患者鞍上下丘脑肿块和皮肤病变中存在 RDD,证实患者在无淋巴结病的情况下同时患有 CNS-RDD 和皮肤-RDD。
认识到 RDD 等罕见疾病的独特表现可防止适当干预和治疗的延迟。