Surgical Pathology Department, Centro Hospitalar de Lisboa Ocidental, Lisboa, Portugal
Dermatology Department, Centro Hospitalar de Lisboa Ocidental, Lisboa, Portugal.
BMJ Case Rep. 2021 Feb 4;14(2):e239244. doi: 10.1136/bcr-2020-239244.
Rosai-Dorfman disease is a rare benign histiocytic proliferative disease of unknown cause that, in exceptional cases, presents with lesions confined to the skin. Clinically variable types of lesions such as papules, nodules and plaques have been reported. We present a case of a 27-year-old woman with a 1-year history of erythematous papular and nodular lesions on the malar and right axillary regions, previously misdiagnosed as acne. She reported no fever, malaise or weight loss, while physical examination and laboratory workup were normal. Bacteriological and mycobacteriological cultures were negative. Histopathological findings showed dense infiltration of inflammatory cells involving the entire dermis, consisting of large macrophages with emperipolesis, S100 and CD68 positive, neutrophils, eosinophils, lymphocytes and plasma cells. The patient was treated with oral prednisolone without improvement. Dapsone was subsequently initiated with favourable clinical response. The present article aimed to emphasise the clinical and histological differential diagnosis and share the treatment experience.
罗萨-多夫曼病（Rosai-Dorfman disease）是一种罕见的良性组织细胞增生性疾病，病因不明，在极少数情况下，病变局限于皮肤。临床上已报道过多种类型的皮损，如丘疹、结节和斑块。我们报告了一例 27 岁女性患者，其右侧腋窝和颧骨部有 1 年的红斑性丘疹和结节性皮损，之前误诊为痤疮。患者无发热、不适或体重减轻，体格检查和实验室检查均正常。细菌和分枝杆菌培养均为阴性。组织病理学检查显示整个真皮层有密集的炎症细胞浸润，其中包括具有胞质包涵体的大型巨噬细胞，S100 和 CD68 阳性，还有中性粒细胞、嗜酸性粒细胞、淋巴细胞和浆细胞。患者口服泼尼松龙治疗，但病情无改善。随后开始使用氨苯砜治疗，取得了良好的临床反应。本文旨在强调该病的临床和组织学鉴别诊断，并分享治疗经验。
