儿童原发性皮肤骨瘤11例临床分析  

Clinical analysis of 11 children with primary osteoma cutis

作　　者：徐教生[1] 向欣[1] 徐子刚[1] 邢嬛[1] 马琳[1] Xu Jiaosheng;Xiang Xin;Xu Zigang;Xing Huan;Ma Lin(Department of Dermatology,Beijing Children′s Hospital,Capital Medical University,National Center for Children′s Health,Beijing 100045,China)

机构地区：[1]首都医科大学附属北京儿童医院皮肤科,100045

出　　处：《中华皮肤科杂志》2019年第8期525-528,共4页Chinese Journal of Dermatology

摘　　要：目的探讨儿童原发性皮肤骨瘤的临床表现、病理特征及转归。方法收集2011—2018年北京儿童医院皮肤科确诊的儿童原发性皮肤骨瘤11例,回顾其临床特点、组织病理学特征及转归。结果11例中,男7例,女4例,均于出生后22个月内发病,中位发病年龄1个月,10例于出生后6个月内起病。皮损表现为大小不等的肤色或淡红色质硬的丘疹、斑块或结节,伴轻度表皮萎缩;3例皮损局限于同一部位,8例累及多个部位。所有患儿血清钙及甲状旁腺激素水平正常,出生时未见畸形。组织病理均可见真皮内成熟板层骨形成,5例合并皮下脂肪组织受累。10例起病8 ~ 18个月后皮损稳定,符合原发性斑块性皮肤骨瘤;1例患儿皮损缓慢持续进展,侵犯皮下深部组织,出现运动障碍,符合进行性骨发育异常。结论儿童原发性皮肤骨瘤好发于婴幼儿时期,临床表现为萎缩性、质硬的皮肤斑块或结节,病理表现为成熟板层骨形成,需要长期随访,警惕进行性骨发育异常。Objective To investigate clinical manifestations, pathological features and outcomes of primary osteoma cutis in children. Methods Eleven children with confirmed primary osteoma cutis diagnosed in Department of Dermatology, Beijing Children′s Hospital between 2011 and 2018 were included into this study. The clinical manifestations, histopathological features, and outcomes were analyzed retrospectively. Results Among the 11 patients, 7 were males and 4 were females. Primary osteoma cutis occurred within 22 months after birth in all the children, the median age of onset was 1 month, and the disease occurred during the first 6 months of life in 10 children. The skin lesions were characterized by skin-colored or reddish indurated papules, plaques or nodules of varying size with slight epidermal atrophy. Three patients had local skin lesions, and 8 had multiple skin lesions. Serum calcium and parathyroid hormone levels were within normal limits in all the children, and no developmental deformity was observed at birth in any of the children. Histopathological examination revealed the formation of mature lamellar bone in the dermis in all the cases, which involved the subcutaneous adipose tissue in 5 cases. The skin lesions became stable 8 - 18 months after the occurrence in 10 patients, which was consistent with primary plaque-like osteoma cutis. Only 1 patient underwent a slowly progressive course, and the skin lesions involved subcutaneous deep tissues, leading to dyskinesia, which was consistent with progressive osseous heteroplasia. Conclusions Primary osteoma cutis in children mostly occurs in infancy, whose clinical manifestations are atrophic, indurated plaques or nodules, and its main pathological feature is the formation of mature lamellar bone. Long-term follow-up is needed, and attention should be paid to the occurrence of progressive osseous heteroplasia.

关 键 词：骨化 异位性 儿童 皮肤骨瘤 原发性 

分 类 号：R739.5[医药卫生—肿瘤]