对称性肢端角化病62例临床分析

目的 分析对称性肢端角化病患者的临床表现、人口学资料、组织病理学特点。方法 收集2003年5月以来在我院门诊就诊的对称性肢端角化病患者62例,详细了解其人口学资料及家族史,并进行全身体格检查,部分患者行皮损组织病理检查。结果 62例患者中男55例,女7例,发病年龄4 ～ 53岁,平均年龄24.02岁;病程15 d至10年,平均为26.65个月;主要临床表现为对称性褐色角化性斑片,皮损接触水后变白,主要分布在指背、手背、手腕、掌侧缘、踝部、膝部、肘部,冬季自然缓解;真菌检查阴性;组织病理学表现为表皮角化过度,棘层增厚,轻度乳头瘤样增生,真皮少量淋巴细胞。结论 对称性肢端角化病以对称性肢端角化为主要临床表现,伴有明显季节性变化。     

疣状肢端角化症的临床及病理特点研究

目的：探讨疣状肢端角化症的临床及病理特点。方法：分析本文及近40年来13篇文献报道的17例肢端角化症先证者的临床及病理资料。结果：发病年龄，10例（59％）〈20岁，7例（41％）〉25岁；有家族史者10例（59％）。皮损主要分布于双手背、足背及前臂、小腿伸侧。皮损表现16例（94％）为扁平疣样损害，1例表现为寻常疣样损害，6例（35％）伴有掌跖角化，5例（29％）伴有指趾甲受累，3例（18％）伴有毛囊角化病。皮损组织病理均表现为表皮明显角化过度、颗粒层及棘层增厚、乳头瘤样增生及塔尖样表皮局限性隆起等特征性变化，无角化不全及空泡化细胞。结论：疣状肢端角化症大部分患者有家族史，常于加岁前发病。皮损主要分布四肢伸侧，基本皮疹为扁平疣样的褐色扁平丘疹。皮损病理显示表皮明显角化过度、乳头瘤样增生及塔尖样表皮局限性隆起等特征性变化，是诊断疣状肢端角化症的主要依据。     

季节性对称性肢端角化症29例临床分析

目的:分析29例对称性肢端角化症患者的临床表现.方法:通过对2003年9月至2008年9月来我所就诊的29例对称性肢端角化症患者的人口学资料、临床表现及病理的分析,总结其临床及病理特征.结果:29例患者起病多为青壮年,皮损特点为对称分布的红褐色角化性斑片(29/29),多发于手腕、手背,遇水皮肤发白,有明显的季节性变化.病理学表现为角化过度、棘层肥厚、真皮浅层血管周围淋巴细胞浸润.结论:本组病例是否为一种新的皮肤病,或是进行性对称性红斑角化的一种亚型有待于进一步的研究.     

六例对称性肢端角化病的透射电镜观察

目的 探讨对称性肢端角化病的超微组织病理特点。方法 收集广东医科大学附属医院皮肤科6例对称性肢端角化病皮损及其周围外观正常皮肤、3例正常人皮肤活检标本,做透射电镜观察。结果 皮损中角质层明显增厚,角化细胞形态不规则,角化包膜不连续;表皮各层角蛋白细丝聚集和排列异常;角质层与颗粒层移行区可见较多大小不等的空泡;颗粒层变薄,透明角质颗粒形态及大小异常,被膜颗粒减少;基底层黑素细胞增多,胞质中有大量Ⅳ期黑素小体;真皮浅层中少数淋巴细胞浸润。皮损周围外观正常皮肤亦有类似超微结构变化,但程度较轻。结论 该病皮损中可能存在角蛋白、表皮分化复合体蛋白及脂质代谢异常,从而导致表皮增厚及屏障功能受损。     

对称性肢端角化病——一种新命名的皮肤病

目的:报告11例以对称性肢端角化为临床表现的患者,对其人口学资料、临床表现、组织病理学特点进行总结,并提出"对称性肢端角化病"这一新的病名.方法:收集2005年5月以来在我所门诊就诊、以手背及手腕出现特征性褐色角化性斑片的患者11例,详细询问个人史及家族史,并进行全身体格检查.部分患者行皮损组织病理检查.结果:11例患者中男9例,女2例,平均年龄35.4岁,平均发病年龄26.5岁;主要临床表现为手背、指背、手腕出现对称性褐色角化性斑片(11/11),主要分布在掌指关节及其周围、指背、手背两侧和手腕屈侧,足背损害较轻,损害有明显的季节性;组织病理学表现为表皮角化过度和轻度乳头瘤样增生.结论:11例患者的临床表现与现有的任何一种皮肤病均不吻合或无法归属于某种皮肤病,因此提出一种新的皮肤病病名--对称性肢端角化病.     

对称性肢端角化病1例

患者男,18岁,双手足角化性斑片2年。表现为手腕、手背及足背对称性褐色角化性斑片,皮损浸水后发白,冬季明显缓解。皮损组织病理示:角化过度,棘层增生,轻度乳头瘤样增生,真皮小血管扩张,真皮浅层血管周围少量淋巴细胞浸润。诊断:对称性肢端角化病。     

表现为色素性对称性肢端角化性皮损的一组患者临床分析

目的 报道一组表现为色素性对称性肢端角化性皮损患者的临床表现、组织病理学及治疗结果。方法 系统地收集了自1998年5月至2007年9月来我科就诊的以色素性对称性肢端角化性皮损为表现的16例患者的临床资料，包括临床表现、皮损组织病理学改变、治疗、性别、年龄、职业、家族成员情况等。结果 男14例，女2例；年龄9 ~ 54岁；病程2个月至8年，平均（2.0 ± 1.5）年。主要临床表现为出现棕褐或棕黑色斑，均呈对称性斑丘疹，表面粗糙，有少许脱屑，伴轻度瘙痒。皮损部位：16例（100%）患者在双手掌指背、手腕，2例（12.5%）双肘及双膝，3例（18.8%）双踝部。组织病理学表现：表皮角化过度，棘层增厚，乳头瘤样增生；真皮少许淋巴细胞浸润。家庭成员无类似病史。给予口服维A酸类、烟酰胺、维生素B6等，外用维A酸软膏、糖皮质激素类软膏等，疗效欠佳，或病情反复。结论 这是一组主要以肢端出现对称性色素性角化斑或斑丘疹，组织病理学表现以表皮角化过度、棘层增厚、乳头瘤样增生为特征的皮肤病，病因不明。     

对称性肢端角化病九例分析

目的 探讨9例对称性肢端角化病患者的相关危险因素、临床表现、治疗及疗效.方法 门诊收集9例对称性肢端角化病患者的临床资料.对皮损进行拍照,真菌镜检、组织病理检查.予外用药治疗并随访.结果 男8例,女1例,平均年龄28.2岁,平均发病年龄24.6岁,病程2个月至16年；皮损对称分布,双手背、腕全部累及,踝次之；手腕部皮损分布呈圈状；夏季重、冬秋季多数自愈；大部分患者与塑胶或橡胶接触有关,部分合并寻常性鱼鳞病；真菌镜检均阴性,组织病理学表现为表皮角化过度,棘层肥厚,轻微乳头瘤样增生；尿素维E乳膏、丙酸氯倍他索乳膏按1∶1调用明显有效.结论 寻常性鱼鳞病患者存在对称性肢端角化病的易患因素,遇水变白可能是水、空气参与的一种物理现象.外用糖皮质激素可作为第一线药物选择.     

角化性皮肤病

20060252疣状肢端角化症的临床及病理特点研究/王培光(安徽医大一附院皮肤科),林国书,高敏…∥中国麻风皮肤病杂志.-2005,21(11).-872~874分析本文及近40年来文献报道的17例肢端角化症先证者的临床及病理资料。发现该症大部分患者有家族史,常于20岁前发病。皮损主要分布于四肢伸侧,基本皮疹为扁平疣样的褐色扁平丘疹。皮损病理显示表皮明显角化过度、乳头瘤样增生及塔尖样表皮局限性隆起等特征性变化,是诊断疣状肢端角化症的主要依据。图2表1参16(赵恩兵)20060253棘状角皮症/刘跃华(中国医科院、协和医大北京协和医院皮肤科),李方凯…∥临床…     

对称性肢端角化病四例

报道4例对称性肢端角化病患者,其中男3例,女1例。表现为手腕、手背及足背部对称性红褐色角化性斑片,遇水皮损发白,冬季明显缓解。组织病理：角化过度,角化不全,表皮增生,棘层增厚,真皮浅层血管周围可见少许淋巴细胞浸润。符合对称性肢端角化病。     

Is acquired symmetrical acrokeratoderma a new dermatosis? Two case reports and Chinese literature review

Background A series of cases characterized by symmetrical acral hyperkeratosis, mainly involving the dorsal surface of the hands, feet, and wrists, but sparing the palmoplantar areas, as well as rapid immersion upon exposure to water have been recently described in China, but similar disorders have not been reported in the English literature. Methods The clinicopathologic features of two cases of acquired symmetrical acrokeratoderma were reported and 27 Chinese patients were reviewed. Results The disorder typically occurred in young or middle aged men. Brown to black hyperkeratotic patches were symmetrically distributed particularly on the wrists and dorsum of hands, fingers, and feet, but without involvement of palms and soles. The lesions became whitish with mild swelling immediately after contact with water and improved generally in winter. Histopathologic examination revealed epidermal hyperkeratosis, acanthosis, and papillomatous hyperplasia, as well as superficial perivascular lymphohistiocytic infiltrate. Main ultrastructural features of the immersed lesion were epidermal hyperkeratosis and spongiosis with partial split of the desmosomes. Conclusion This disorder may be a new dermatosis, and the term “acquired symmetrical acrokeratoderma” could exactly reflect its clinicopathologic characteristics.     

对称性肢端角化病一例

患者,男,16岁。双手角化性斑片3年、复发2个月。本患者与既往报道的SAK病例不同,初期仅表现为手掌及指侧缘淡黄色角化性斑片,随着病情进展,皮损表现为黑褐色角化性斑片,皮损浸水后均发白、起皱,伴轻微紧绷感,离开水后恢复原状。治疗1个月后随访,皮损基本消退。     

Expression of keratins 14, 10 and 16 in marginal keratoderma of the palms

Marginal keratoderma of the palms (MKP), also known as degenerative collagenous plaques of the hands, is an infrequent disease affecting individuals with photoaged skin and submitted to mechanical trauma of the hands. The aim of the study was to study the expression of keratin 14 (K14) in the basal layer and keratins 10 (K10) and 16 (K16) in the suprabasal layer, and to establish the effect of growth factors and receptors of the epidermal growth factor in the genesis of hyperkeratosis observed in MKP. The study included 14 patients with MKP. Expression of keratins 10, 14 and 16 was visualized by immunohistochemical staining using monoclonal antibodies by standard immunoperoxidase method. As control three normal skin samples were used. Absence or weak expression of keratins 14 and 10, and strong expression of keratin 16 was recorded in MKP patients. It was concluded that MKP patients present weak expression of keratins 14 (basal membrane) and 10 (suprabasal layer) but strong expression of keratin 16, emphasizing the importance of stimulation of epidermal growth factor receptors by ultraviolet radiation and traumatism.     

Hereditary papulotranslucent acrokeratoderma: a case report and literature review

Hereditary papulotranslucent acrokeratoderma is a rare autosomal-dominant syndrome of the hands and feet characterized by persistent, asymptomatic, yellowish to white papules and plaques associated with fine-textured scalp hair and an atopic diathesis. Histopathologically, focal hyperkeratosis, hypergranulosis, and acanthosis of the epidermis are seen. We present a case of hereditary papulotranslucent acrokeratoderma in a young adult woman. The literature of this unusual condition is reviewed, and its relationship to acquired papulotranslucent acrokeratoderma is discussed.     

Aquagenic palmoplantar keratoderma

Aquagenic palmoplantar keratoderma is an acquired condition characterized by burning and edema limited to the hands after brief immersion in water. The 3 patients described possess a striking similarity to those with transient reactive papulotranslucent acrokeratoderma. All 3 patients manifested the "hand-in-the-bucket" sign, having presented to a physician with a hand immersed in a bucket of water to more promptly demonstrate the physical findings. Aluminum chloride hexahydrate represents a potentially valuable therapeutic option for this unusual condition.     

二维电泳和质谱技术筛选寻常型银屑病皮损差异表达蛋白

目的:明确寻常型银屑病(psoriasis vulgaris,PV)皮损组织与正常人皮肤中的差异表达蛋白。方法:收集PV皮损组织及正常人皮肤组织。提取蛋白,进行二维凝胶电泳,选择在PV皮损组织中明显差异表达的蛋白点,进行质谱和生物信息学分析。结果:PV患者角质层和真皮层二维凝胶电泳鉴别蛋白总数分别为(1961±21)和(1928±49)个,确定差异表达蛋白30个,其中14个被鉴定分型:包括角质1型细胞骨架(K1C10、K1C14、K1C15、K1C16)、角质2型细胞骨架(K2C1),肌动蛋白(ARP3,ACTA,ACTBM),热休克蛋白(PHB,HSPβ1,CH60),中心体蛋白(CP135),膜联蛋白(ANXA4、ANXA5)。结论:差异表达蛋白可能在PV的病理形成中起重要作用,是潜在的PV诊断标记物。     

Long-term alteration in the expression of keratins 6 and 16 in the epidermis of mice after chronic UVB exposure

The influences of chronic UVB exposure on epidermal differentiation have been poorly studied compared to dermal photo-aging although those effects are very important in terms of photo-damage to the skin. The purpose of this study was to investigate the effects of chronic UVB exposure on keratin expression in the epidermis. The effects on murine skin of chronic exposure to weak UVB (below 1 MED) was examined by immunoblotting for keratins K10, K5, K6, and K16, by immunohistochemistry using antibodies to K6, K16, and Ki67 as well as by conventional HE staining of skin sections. Alterations of keratin expression induced by the chronic UVB exposure were distinct from those elicited by a single acute UVB exposure. The expression of keratins K6 and K16 was quite long-lasting, continuing for 7 weeks after 6 weeks of chronic UVB exposure and for 6 weeks after 9 weeks of chronic UVB exposure. In contrast, K6 and K16 expression induced by a single UVB exposure at 0.5 MED or 3 MED almost ceased within 2 weeks after that exposure. Furthermore, the expression of the constructive keratins, K5 and K10, remained almost unchanged by chronic UVB exposure. Epidermal thickness was increased significantly immediately after the 9 weeks of chronic UVB exposure; however, it had returned to normal level 6 weeks later. The alterations in keratin expression accompanied the marked disruption of the ordered ultrastructure of keratin intermediate filaments, which were observed by TEM. Thus, chronic exposure to UVB has a deep impact on the biosynthetic regulation of different keratins in the epidermis, thereby interfering with the ordered ultrastructure of keratin intermediate filaments. Those events could have relevance to the mechanism of photo-damage, such as fine wrinkles observed in chronically UV-exposed skin in addition to dermal photo-aging.