Department of Pediatrics, Wonkwang University Sanbon Medical Center, Gunpo, Korea.
J Korean Med Sci. 2012 Jan;27(1):96-100. doi: 10.3346/jkms.2012.27.1.96. Epub 2011 Dec 19.
The triad of rash, arthritis, and uveitis seems to be characteristic for early-onset childhood sarcoidosis. We describe an interesting case of early-onset childhood sarcoidosis coexisting enchondromatosis, which clinically masquerade as Langerhans cell histiocytosis. A 33 months old girl presented with skin rash, subcutaneous nodules with polyarthritis, and revealed the involvement of lymph nodes as well as spleen during work-up. She also presented with multiple osteolytic lesions which pathologically proven enchondromatosis. Oral prednisone was prescribed at 2 mg/kg/day for 2 months until when subcutaneous nodules and joint swellings almost disappeared, and then slowly tapered over a period of 5 months. We report an unusual case of early-onset childhood sarcoidosis presented with osteolytic bone lesions which were irrelevant to sarcoidosis.
皮疹、关节炎和葡萄膜炎三联征似乎是儿童发病早发性结节病的特征。我们描述了一例有趣的早发性儿童结节病合并软骨瘤病的病例，临床上表现为朗格汉斯细胞组织细胞增生症。一名 33 个月大的女孩出现皮疹、皮下结节伴多发性关节炎，并在检查过程中显示淋巴结和脾脏受累。她还出现了多个溶骨性病变，经病理证实为软骨瘤病。给予口服泼尼松 2mg/kg/天治疗 2 个月，直到皮下结节和关节肿胀几乎消失，然后在 5 个月内逐渐减量。我们报告了一例不常见的早发性儿童结节病病例，其表现为与结节病无关的溶骨性骨病变。
