Snoussi Mouna, Derbel Abir, Hentati Yosr, Mnif Zeineb, Marzouk Sameh, Bahloul Zouheir
Department of Internal Medicine, Hedi Chaker Hospital, Sfax, Tunisia.
Department of Medical Imaging, Hedi Chaker Hospital, Sfax, Tunisia.
Eur J Case Rep Intern Med. 2023 Feb 1;10(1):003389. doi: 10.12890/2023_003389. eCollection 2023.
Rosai-Dorfman disease (RDD) is an uncommon lymphoproliferative disorder; RDD with oropharyngeal involvement is extremely rare, especially in adults. A 65-year-old woman with a complaint of progressive dyspnoea since 2016 presented with laryngeal involvement of RDD. A laryngoscopy examination revealed two solid, polypoid masses in the subglottic region, and a laryngeal biopsy concluded chronic inflammation without signs of malignancy. A second biopsy of axillary lymph nodes was performed, supporting the diagnosis of histiocytosis. The patient was treated with corticosteroids and then lost to follow-up. In 2019, she suffered from dyspnoea and a hoarse voice. Laryngoscopy examination showed a polypoid lesion causing airway obstruction at 70% and thickening of the lateral wall of the cavum. Physical examination found left axillary and submandibular adenopathy, and computed tomography revealed thickening of the supraglottic larynx narrowing the laryngeal pathway. Lymphadenectomy with immunohistochemical analysis revealed typical protein positive S-100 histiocytes and emperipolesis. The patient was treated with high doses of corticosteroids for six weeks then these were progressively decreased. The outcome was favourable; the laryngeal lesion disappeared after two weeks of treatment.
Rosai-Dorfman disease is a rare cause of lymphadenopathy in adults. Extranodal presentation of the disease is possible mainly in the head and the neck region.The diagnosis is based on histological examination with the presence of histiocytes, which are S-100 positive, CD68 positive, and CD1a negative immunohistochemistry.The outcome is usually good in asymptomatic forms of the disease with no critical organ involved. The surgical resection is appropriate to the localised symptomatic form of the disease while corticosteroids are indicated in disseminated RDD as a first-line therapy.Inspired by our case, rare localisation of Rosai-Dorfman disease (RDD), led to clinical and therapeutic issues. That is why a review of the literature must be undertaken, to share experiences.
罗萨伊-多夫曼病（RDD）是一种罕见的淋巴增生性疾病；累及口咽的RDD极为罕见，尤其是在成人中。一名65岁女性自2016年以来出现进行性呼吸困难，表现为RDD累及喉部。喉镜检查显示声门下区域有两个实性、息肉样肿物，喉部活检结果为慢性炎症，无恶性迹象。对腋窝淋巴结进行了第二次活检，支持组织细胞增多症的诊断。患者接受了皮质类固醇治疗，随后失访。2019年，她出现呼吸困难和声音嘶哑。喉镜检查显示一个息肉样病变导致气道阻塞70%，并伴有喉腔侧壁增厚。体格检查发现左腋窝和下颌下淋巴结肿大，计算机断层扫描显示声门上喉增厚，使喉道变窄。淋巴结切除并进行免疫组化分析显示典型的S-100蛋白阳性组织细胞和血细胞吞噬现象。患者接受了六周的高剂量皮质类固醇治疗，然后逐渐减量。结果良好；治疗两周后喉部病变消失。
罗萨伊-多夫曼病是成人淋巴结病的罕见病因。该病的结外表现主要见于头颈部区域。诊断基于组织学检查，存在S-100阳性、CD68阳性且CD1a阴性免疫组化的组织细胞。对于无症状且无重要器官受累的疾病形式，预后通常良好。对于局限性有症状的疾病形式，手术切除是合适的，而对于播散性RDD，皮质类固醇作为一线治疗药物。受我们病例的启发，罗萨伊-多夫曼病（RDD）的罕见定位导致了临床和治疗问题。这就是为什么必须进行文献综述以分享经验。
