传染性软疣样表现的先天自愈性朗格汉斯细胞组织细胞增生症5例皮肤镜特点及临床与组织病理分析

Dermatoscopic features and clinicopathological analysis of 5 patients with congenital self-healing Langerhans cell histiocytosis presenting as molluscum contagiosum

目的:总结5例传染性软疣样表现的先天自愈性朗格汉斯细胞组织细胞增生症临床特征、皮肤镜下表现及组织病理学特点。方法:采用回顾性分析对昆明医科大学附属昆明市儿童医院皮肤科5例传染性软疣样表现的先天自愈性朗格汉斯细胞组织细胞增生症患儿的临床资料、皮肤镜图片、组织病理及免疫组化染色资料进行分析总结。结果:5例患儿中,男3例,女2例,平均发病月龄为4.5个月,平均病程2.1个月。所有患儿皮肤镜下表现有2种,丘疹区在皮肤镜偏振光下可见淡红色及肤色类圆形结构区,部分中央见云雾状黄白色或褐色鳞屑,浸润法下边缘红色背景褪色呈白色环。白色萎缩区在皮肤镜偏振光下可见边界清楚的白色背景中央紫红色或褐色结构区。浸润法下中央紫红色或褐色结构区不能褪色。组织病理学表现为弥漫性浸润或灶性浸润的淋巴细胞、组织细胞及嗜酸性粒细胞,并可见胞质丰富、淡伊红染色的单核样细胞,核呈椭圆形或肾形,部分可见核沟。免疫组化中朗格汉斯细胞组织细胞S-100蛋白、CD1a、Langerin均阳性。结论:该病临床少见,容易被误诊,结合皮肤镜下特点可初步判断,提高病检率,依靠组织病理学及免疫组织化学染色进行确诊,该病有可能转化为系统性朗格汉斯细胞组织细胞增生症,需长期随访。

Objective: To study the clinical manifestations, dermatoscopic features and histopathological characteristics of 5 patients with congenital self-healing langerhans cell histiocytosis presenting clinically as molluscum contagiosum. Method: We performed a retrospective analysis of the data, including clinical manifestations, dermatoscopic features, histopathological and immunohistochemical findings of 5 children with congenital self-healing langerhans cell histocytosis presenting clinically with molluscum contagious in Department of Dermatology of Kunming Children’s Hospital, the Affiliated Hospital of Kunming Medical University. Result: Among these 5 cases, 3 were male and 2 were female. The average age of onset of disease was 4.5 months, and the average course of the disease was 2.1 months. All the children had two common changes under the dermoscopy: the papule area and the white atrophy area.The papule areas had the circular like structure with the pink and skin color under the polarized light of dermoscopy. The center of some papules had cloudy, yellow-white or brown scales. In the white atrophy area, there was a purplish red or brown structure in the center of a well-circumscribed, white background under polarized light of the dermoscopy. The purplish red or brown structure in the center did not fade when the infiltrating method was applied. Histopathological features include a diffuse histiocytic infiltrate mixed with a few lymphocytes and eosinophils in the dermis. There are mononuclear cells with abundant eosinophilic cytoplasm and oval to kidney-shaped nuclei, some of which were grooved. Most dermal infiltrates were positive for S-100, CD1 a and Langerin, consistent with langerhans cells. Conclusion: Congenital self-healing langerhans cell histiocytosis is a rare form of langerhans cell histiocytosis, which can be easily misdiagnosed. The dermatoscopic characteristics would be helpful to improve its early diagnosis. Its diagnosis can be confirmed by histopathology and immunohistochemistry. A long-term follow-up is necessary.