朗格汉斯细胞组织细胞增多症与罗萨伊-多夫曼病罕见并存：一例报告及文献复习
An unusual co-occurrence of Langerhans cell histiocytosis and Rosai-Dorfman disease: report of a case and review of the literature.
作者信息
Cohen-Barak Eran, Rozenman Dganit, Schafer Jan, Krausz Judith, Dodiuk-Gad Roni, Gabriel Hertzel, Shani-Adir Ayelet
机构信息
Department of Dermatology, Haemek Medical Center, Afula, Israel.
出版信息
Int J Dermatol. 2014 May;53(5):558-63. doi: 10.1111/ijd.12051. Epub 2013 Aug 22.
BACKGROUND
The co-occurrence of Langerhans cell histiocytosis (LCH; disorder characterized by proliferation of Langerhans cells) and Rosai-Dorfman disease (RDD; histiocytic entity that is one of the non-Langerhans cell diseases) is extremely rare and raises several questions regarding the nature of the diseases.
METHODS
We describe a 10-year-old boy who presented with a 3-month history of right scalp swelling. Clinical, pathological, and imaging evaluation disclosed multiple LCH of bone and cutaneous RDD.
RESULTS
After initiating prednisone and vinblastine therapy, the patient developed an asymptomatic diffuse papular eruption. Biopsy revealed cutaneous RDD. Treatment was continued with a good response of bone LCH and significant amelioration of the cutaneous findings.
CONCLUSIONS
Co-occurrence of LCH and RDD is a rare phenomenon. Various explanations, including the role of chemotherapy, are suggested based on our and several previously reported cases.
背景
朗格汉斯细胞组织细胞增多症（LCH；以朗格汉斯细胞增殖为特征的疾病）与罗萨伊-多夫曼病（RDD；非朗格汉斯细胞疾病之一的组织细胞性病变）同时出现极为罕见，引发了关于这些疾病本质的若干问题。
方法
我们描述了一名10岁男孩，他有3个月的右头皮肿胀病史。临床、病理和影像学评估显示存在骨多发性LCH和皮肤RDD。
结果
在开始使用泼尼松和长春碱治疗后，患者出现无症状的弥漫性丘疹性皮疹。活检显示为皮肤RDD。继续治疗后，骨LCH有良好反应，皮肤表现明显改善。
结论
LCH和RDD同时出现是一种罕见现象。基于我们的病例以及先前报道的若干病例，提出了各种解释，包括化疗的作用。
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