Department of Dermatology, Tianjin Children's Hospital, Tianjin, China.
Department of Stomatology, Tianjin Medical University General Hospital, Tianjin, China.
Br J Dermatol. 2018 Jan;178(1):261-264. doi: 10.1111/bjd.15306. Epub 2017 Sep 26.
Erdheim-Chester disease (ECD), a type of systemic non-Langerhans cell histiocytosis, is uncommon and characterized by the accumulation of CD68 CD1a foamy histiocytes. It is extremely rare in children. The skin lesions of paediatric ECD have not been systemically described before. We report a case of ECD in a 3·5-year-old Chinese boy. The patient presented with generalized skin and bone involvement of 3 years' duration. Marked generalized annular maculopapular lesions with central atrophy were seen. These differed from previously reported adult xanthoma-like papules or periorbital xanthelasma-like lesions. Computed tomography revealed diffuse pulmonary fibrosis and generalized skeletal involvement, including osteolysis and osteosclerosis. The presence of CD68 CD1a histiocytes allowed the diagnosis of ECD. According to our review of the literature, this is the paediatric case of ECD with the youngest age of onset. The generalized skin involvement made our case unique in comparison with those previously reported.
厄尔-当-切斯特病（ECD），一种系统性非朗格汉斯细胞组织细胞增生症，较为罕见，其特征是 CD68 CD1a 泡沫样组织细胞的积累。在儿童中极为罕见。儿科 ECD 的皮肤病变以前没有系统描述过。我们报告了一例 3.5 岁中国男孩的 ECD。患者表现为全身性皮肤和骨骼受累，病程 3 年。可见广泛的环形斑丘疹，中央萎缩。这些与以前报道的成人黄色瘤样丘疹或眶周黄色瘤样病变不同。计算机断层扫描显示弥漫性肺纤维化和全身性骨骼受累，包括溶骨性和骨硬化性病变。CD68 CD1a 组织细胞的存在有助于 ECD 的诊断。根据我们对文献的回顾，这是发病年龄最小的儿科 ECD 病例。与以前报道的病例相比，全身性皮肤受累使我们的病例具有独特性。
