本文選題：板樣皮膚骨瘤　切入點(diǎn)：原發(fā)性　出處：《臨床皮膚科雜志》2017年10期

【摘要】：目的:探討原發(fā)性皮膚骨瘤(plate-like osteoma cutis,PLOC)的臨床表現(xiàn)、組織病理特點(diǎn)及診斷要點(diǎn)。方法:回顧性分析1例板樣PLOC的臨床及組織病理資料�；純耗�,3歲。出生后3個(gè)月發(fā)現(xiàn)腹部、肩胛部、下肢皮膚多發(fā)性大小不等的板狀皮損,質(zhì)硬如骨。沒(méi)有特殊個(gè)人史或家族史,也沒(méi)有鈣磷代謝異常及甲狀旁腺激素或甲狀腺功能異常。皮損自患兒6個(gè)月后至今已3年,病情無(wú)進(jìn)展。結(jié)果:皮損組織病理檢查示表皮正常,真皮及皮下脂肪組織內(nèi)局灶性分布板層狀骨組織,骨組織中見(jiàn)骨細(xì)胞,部分骨小梁周圍可見(jiàn)成骨細(xì)胞,診斷為皮膚骨瘤。結(jié)論:PLOC是罕見(jiàn)的原發(fā)性皮膚骨瘤,是非漸進(jìn)性的異位骨化;其與進(jìn)行性骨發(fā)育異常(progressive osseus heteroplasia,POH)和Albright遺傳性骨營(yíng)養(yǎng)不良(Albright hereditary osteodystrophythe,A HO)均屬于GNAS基因相關(guān)性骨化障礙性疾病。
[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.
【作者單位】： 山西省兒童醫(yī)院皮膚科;
【分類號(hào)】：R739.5

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