Division of Pediatric Rheumatology, Penn State Milton S Hershey Medical Center, Hershey, Pennsylvania, USA.
Pediatr Rheumatol Online J. 2009 Oct 8;7:15. doi: 10.1186/1546-0096-7-15.
Xanthomatous skin lesions and arthritis in children are not a common association. We present the case of a 3 year old girl who presented with xanthomatous lesions in the periungual region of both hands, around the nares and on her forehead, associated with significant arthritis that was clinically compatible with multicentric reticulohistiocytosis. However, pathology of the xanthomatous lesions was more suggestive of papular xanthoma, a disease that is not associated with arthritis. Based on her presentation and the negative lipid workup, she was treated for presumed multicentric reticulohistiocytosis. Multiple treatment strategies were utilized, with improvement on a combination of infliximab, methotrexate, and prednisone. We review the different diagnoses that should be considered in children with xanthomas and arthritis as well as the different pharmacologic therapies used in children with multicentric reticulohistiocytosis.
儿童的黄色瘤性皮肤病变和关节炎并不常见。我们报告了一例 3 岁女孩的病例，她的双手甲沟周围、鼻翼周围和前额出现黄色瘤性病变，同时有关节炎，临床上符合多发性骨化性纤维结构不良。然而，黄色瘤病变的病理更提示丘疹性黄色瘤，这是一种与关节炎无关的疾病。根据她的表现和阴性脂质检查结果，她被诊断为疑似多发性骨化性纤维结构不良，给予英夫利昔单抗、甲氨蝶呤和泼尼松联合治疗。我们回顾了儿童黄色瘤和关节炎的不同诊断，以及儿童多发性骨化性纤维结构不良的不同药物治疗策略。
