板样皮肤骨瘤1例及临床特征分析
本文选题:板样皮肤骨瘤 切入点:原发性 出处:《临床皮肤科杂志》2017年10期
【摘要】:目的:探讨原发性皮肤骨瘤(plate-like osteoma cutis,PLOC)的临床表现、组织病理特点及诊断要点。方法:回顾性分析1例板样PLOC的临床及组织病理资料。患儿男,3岁。出生后3个月发现腹部、肩胛部、下肢皮肤多发性大小不等的板状皮损,质硬如骨。没有特殊个人史或家族史,也没有钙磷代谢异常及甲状旁腺激素或甲状腺功能异常。皮损自患儿6个月后至今已3年,病情无进展。结果:皮损组织病理检查示表皮正常,真皮及皮下脂肪组织内局灶性分布板层状骨组织,骨组织中见骨细胞,部分骨小梁周围可见成骨细胞,诊断为皮肤骨瘤。结论:PLOC是罕见的原发性皮肤骨瘤,是非渐进性的异位骨化;其与进行性骨发育异常(progressive osseus heteroplasia,POH)和Albright遗传性骨营养不良(Albright hereditary osteodystrophythe,A HO)均属于GNAS基因相关性骨化障碍性疾病。
[Abstract]:Objective: to investigate the clinical manifestations, histopathological features and diagnostic features of primary cutaneous osteoma, plate-like osteoma cutis (PLOC). Methods: the clinical and histopathological data of a case with plate-like PLOC were retrospectively analyzed. The male was 3 years old and the abdomen was found 3 months after birth. Platelike lesions of varying sizes in the skin of the scapula and lower extremities, as hard as bone. No special personal or family history, There was no abnormal calcium and phosphorus metabolism, parathyroid hormone or thyroid dysfunction. The lesions had been 3 years after 6 months and had no progress. Results: the skin lesions showed normal epidermis. In dermis and subcutaneous adipose tissue, osteoblasts were found in lamellar bone tissue, osteoblasts were found in bone tissue, and osteoblasts were found around trabeculae, which were diagnosed as cutaneous osteomas. Nonprogressive heterotopic ossification, which is associated with progressive osseus heteroplasia (POH) and Albright hereditary bone dystrophy (Albright hereditary osteodystrophyrophytophysis A), belongs to GNAS gene-related ossification disorder.
【作者单位】: 山西省儿童医院皮肤科;
【分类号】:R739.5
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