Touati Med Dheker, Omry Ahmed, Ferjaoui Wael, Haloui Nabil, Gargouri Faten, Khalifa Med Bachir
General Surgery Department, Military Hospital of Tunis, Mont Fleury-1008, Tunis, Tunisia; Faculty of Medicine of Tunis, 15, Djebel Lakhdhar Street - 1007 Bab Saadoun, Tunis, Tunisia.
General Surgery Department, Military Hospital of Tunis, Mont Fleury-1008, Tunis, Tunisia; Faculty of Medicine of Tunis, 15, Djebel Lakhdhar Street - 1007 Bab Saadoun, Tunis, Tunisia.
Int J Surg Case Rep. 2024 Apr;117:109475. doi: 10.1016/j.ijscr.2024.109475. Epub 2024 Mar 5.
Rosai-Dorfman disease (RDD) is a rare disorder characterized by benign histiocytic proliferation. The purely cutaneous variant of Rosai-Dorfman disease is exceptionally uncommon. This abstract centers on an extraordinary case-an instance of primary cutaneous Rosai-Dorfman disease (PCRDD), a rare cutaneous variant within an already infrequent disorder. Successfully managed through surgical intervention, this unique case underscores the pressing need for refined diagnostic and therapeutic strategies.
We present a noteworthy case of primary cutaneous Rosai-Dorfman disease (PCRDD) in a north African female, deviating from the typical age of onset. The patient exhibited a rare cutaneous variant, emphasizing the need for heightened awareness in diverse demographics. Histological analysis revealed characteristic emperipolesis and immunohistochemical features consistent with RDD.
PCRDD, although uncommon within the Rosai-Dorfman Disease spectrum, manifests with a later onset and distinctive demographic characteristics. Surgical excision emerges as a viable therapeutic option for managing symptomatic lesions, as demonstrated in our case. The infrequency of PCRDD underscores the importance of tailored diagnostic and therapeutic strategies. Individualized approaches become pivotal in addressing the complexities associated with PCRDD, ensuring careful consideration of its intricacies in the broader context of Rosai-Dorfman disease.
This case underscores the complexity of RDD, especially in rare cutaneous presentations. Surgical excision, with its reported efficacy, stands as a crucial intervention, offering both symptomatic relief and potential cure, showcasing the significance of individualized care in managing this rare disorder. Continued research remains imperative for advancing RDD understanding and refining clinical approaches.
罗萨伊-多夫曼病（RDD）是一种以良性组织细胞增生为特征的罕见疾病。罗萨伊-多夫曼病的纯皮肤型极为罕见。本摘要聚焦于一个特殊病例——原发性皮肤罗萨伊-多夫曼病（PCRDD），这是一种在本就罕见的疾病中的罕见皮肤变异型。通过手术干预成功治疗了该病例，凸显了制定精准诊断和治疗策略的迫切需求。
我们报告了一例北非女性原发性皮肤罗萨伊-多夫曼病（PCRDD）的显著病例，其发病年龄偏离了典型情况。该患者表现出一种罕见的皮肤变异型，强调了在不同人群中提高认识的必要性。组织学分析显示了特征性的组织细胞内吞现象以及与RDD一致的免疫组化特征。
PCRDD在罗萨伊-多夫曼病谱系中虽不常见，但起病较晚且具有独特的人口统计学特征。如我们的病例所示，手术切除是治疗有症状病变的可行治疗选择。PCRDD的罕见性凸显了量身定制诊断和治疗策略的重要性。个性化方法对于应对与PCRDD相关的复杂性至关重要，确保在罗萨伊-多夫曼病的更广泛背景下仔细考虑其复杂性。
该病例凸显了RDD的复杂性，尤其是在罕见的皮肤表现中。手术切除因其已报道的疗效，是一项关键干预措施，既能缓解症状又有潜在治愈可能，展示了个性化护理在管理这种罕见疾病中的重要性。持续研究对于增进对RDD的理解和完善临床方法仍然至关重要。
