CAJ | 학술논문

目的：对16例季节性对称性肢端角化症患者进行临床及病理超微结构分析，归纳总结其特点。方法：追踪随访并收集我所皮肤科门诊2010年3月至2012年11月16例季节性对称性肢端角化症患者的临床资料，对比治疗前后患者皮损的病理及超微结构的改变。结果：16例患者中男11例，女5例，平均年龄（28．3±2．1）岁，平均发病年龄（19.5±1．2）岁。通常春末夏初发病或复发，夏季加重，秋末冬初减轻或消失；皮损主要对称性分布于手腕、手背、踝部、膝部、足背、肘部、指蹼、手侧缘、肘窝，表现为色素性苔藓样斑片或斑块，无明显不适；真菌学检查阴性；组织病理学表现为表皮角化过度和乳头瘤样增生；超微结构显示角质层层数增多，角质化包膜连续性差，角蛋白丝结构紊乱成撕裂状，颗粒层及棘层上部细胞核周围可见异常聚集的角蛋白微丝。结论：本病发病有明显的季节特点，病理及超微结构类似于大疱性鱼鳞病样红皮病，其发病机制可能与角蛋白代谢相关。
목적：대16례계절성대칭성지단각화증환자진행림상급병리초미결구분석，귀납총결기특점。방법：추종수방병수집아소피부과문진2010년3월지2012년11월16례계절성대칭성지단각화증환자적림상자료，대비치료전후환자피손적병리급초미결구적개변。결과：16례환자중남11례，녀5례，평균년령（28．3±2．1）세，평균발병년령（19.5±1．2）세。통상춘말하초발병혹복발，하계가중，추말동초감경혹소실；피손주요대칭성분포우수완、수배、과부、슬부、족배、주부、지복、수측연、주와，표현위색소성태선양반편혹반괴，무명현불괄；진균학검사음성；조직병이학표현위표피각화과도화유두류양증생；초미결구현시각질층층수증다，각질화포막련속성차，각단백사결구문란성시렬상，과립층급극층상부세포핵주위가견이상취집적각단백미사。결론：본병발병유명현적계절특점，병리급초미결구유사우대포성어린병양홍피병，기발병궤제가능여각단백대사상관。
Objective:To analyze the clinical data of 16 patients with seasonal acquired sym-metric acrokeratoderma and summarize its features of clinical manlfestation and ultrastructure . Methods:16 patients in our hospital from March 2010 to November 2012 were followed up and their clinical data were collected and analyzed in the study .Results:There were 11 male and 5 fe-male patients among these 16 patients.The mean age was (28.3 ±2.1) years old and the age at onset was (19.5 ±1.2) years old.Lesions usually occurred or relapsed in the end of spring or the beginning of summer , most of cases were aggravated in summer ,and vanished or alleviated in the end of autumn or the beginning of winter .The lesions usually symmetrically distributed on the both of wrists,the back of hands,ankles,knees,dorsum of feet,elbows,finger webs,lateral margin of hands and triangles of elbow .All lesions showed the appearance of pigmentation ,lichenoid ,hyper-keratotic patches or plaques and without noticeable discomfort on the lesions .KOH tests were neg-ative.The histopathology revealed hyperkeratosis and papillomatosis of the epidermis .Ultrastructure showed cutin layer upon and increased in the layer number , keratinization coated poor continuity , keratin filaments was disorder in structure into tear .The surrounding granular layer and upper spi-nous layer cells , some keratin filaments were visible aggregation abnormally .Conclusion:16 patients with seasonal acquired symmetric acrokeratoderma present obvious seasonal characteristics .Histopa-thology and ultrastructure findings are similar to congenital bullous ichthyosiform erythroderma .