幼儿着色性干皮病伴角化棘皮瘤的临床特征分析  

Analysis of Clinicopathology Features of Xeroderma Pigmentosum Associated with Keratoacanthoma

作　　者：郑金锋[1] 周露婷[1] 王丽[2] 耿明[1] 刘翠杰[2] 

机构地区：[1]济南军区总医院病理科,250031 [2]济南军区总医院皮肤科,250031

出　　处：《中华妇幼临床医学杂志（电子版）》2014年第2期98-101,共4页Chinese Journal of Obstetrics & Gynecology and Pediatrics(Electronic Edition)

摘　　要：目的探讨幼儿着色性干皮病(XP)伴角化棘皮瘤的临床表现、组织病理学特征、诊断、治疗及鉴别诊断。方法以2013年6月6日在济南军区总医院确诊的1例幼儿XP伴角化棘皮瘤患儿为研究对象(本研究遵循的程序符合济南军区总医院人体试验委员会制定的伦理学标准,得到该委员会批准,并征得受试对象监护人的知情同意,并与之签署临床研究知情同意书)。对其罹患的XP伴角化棘皮瘤进行组织病理学检查,并结合相关文献总结该病的临床表现、组织病理学特征、诊断和治疗方法等。结果本例患儿为生后18个月龄,男性,临床表现为面部可见大小不等、形状不规则的褐色色素沉着斑及色素脱失斑,左眼下眼睑下方可见半球形肿物,大小约为3cm×3cm,表面尚光滑,伴褐色斑片,边界清楚,质韧。术后切除皮损组织送组织病理学检查结果示,表皮角化过度,真皮浅层慢性炎性细胞浸润,基底层黑色素细胞不规则增多,黑色素增加。左眼下眼睑下方切除肿物于镜下呈火山口状,其内充满角化物,2侧边缘表皮增生呈唇状向中央包绕,底部表皮呈假上皮瘤样增生,形成不规则的上皮团块及角化物,肿物边界清晰,周围间质内炎性细胞浸润明显。经过手术及药物治疗后,电话随访8个月示患儿恢复良好。结论幼儿XP伴角化棘皮瘤十分罕见。采用避光+抗氧化药物+肿瘤切除综合治疗可取得较满意的疗效。Objective To investigate the clinicopathological characteristics,diagnosis,differential diagnosis and treatment of xeroderma pigmentosum（XP）associated with keratoacanthoma.Methods One case of child （boy,18-month old）with XP associated with keratoacanthoma in Jinan Military General Hospital was reported.The study protocol was approved by the Ethical Review Board of Investigation in Jinan Military General Hospital.Informed consent was obtained from parents of this child.The clinical manifestations,histological features,treatment and prognosis of the disease were analyzed and summarized. Results A 1 8-month old boy was admitted with unequal size,irregularly shaped brown spots,patches and depigmentation spots.A well-circumscribed hemispherical mass measuring 3 cm ＆#215; 3 cm was found with smooth surface and brown patches beneath the left lower eyelid.The microscopical features of skin lesion revealed epidermal hyperkeratosis,chronic inflammatory infiltration in the superficial dermal,and increases of melanocyte and melanin in basal layer.The scanning magnification of the mass beneath the left lower eyelid showed a cup-shaped,squamous proliferation with a central keratin plug.The squamous epithelium was acanthotic with hypergranulosis.The adjacent epidermis formed exophytic projections resulting in a silhouette that has been likened to lips.There was an associated inflammatory reaction within the stroma surrounding the mass.A eight-month follow-up showed a good prognosis.Conclusions XP of infancy is a rare disease,and it is more rare which associated with keratoacanthoma.Combined therapy of antioxidant drugs away from light and excision of the mass remain the treatment of choice.

关 键 词：干皮病 着色性 角化棘皮瘤 病理学 临床 儿童 

分 类 号：R739.5[医药卫生—肿瘤]