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鱼鳞病的病因有哪些?鱼鳞病能治好吗?

发布时间:2016-01-07   问大夫  责任编辑:詹敏怡

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皮肤科的诸多疾病中,鱼鳞病的出现就是属于其中之一。鱼鳞病的症状主要表现为皮肤干燥,尤其是四肢躯干表现的特别严重。当患有了鱼鳞病之后,找对治疗的方式也是很重要的。鱼鳞病的病因有哪些?鱼鳞病能治好吗?下面看看吧。

鱼鳞病

鱼鳞病俗称(蛇皮癣),70%属遗传(遗传病),多于儿童时发病,好发部位四肢躯干仰面,皮肤干燥粗糙,皮屑边缘略翘起,状似蛇皮,或汗毛孔有颗粒物堵塞,长不出汗毛,重者皮肤呈灰褐色鳞屑和深重斑纹,随着年龄增长波及全身,本病特点冬重夏轻。多系遗传因素致表皮细胞增殖和分化异常,导致细胞增殖增加和(或)细胞脱落减少。

鱼鳞病的病因有哪些?

1、表皮松解性角化过度鱼鳞病:

为常染色体显性遗传病。致病基因与角蛋白1(K1)和角蛋白10(K10)基因突变有关,导致角蛋白的合成或降解缺陷,影响基底层角质形成细胞内张力微丝的正常排列与功能,进而造成角化异常及表皮松解。

2、板层状鱼鳞病:

系常染色体隐性遗传。基因定位多个位点,包括2q33-35、19p12-q12、定位于14q11TGM1基因发生突变、缺失或插入,至细胞粘连和细胞被膜蛋白交联缺陷。

3、寻常型鱼鳞病:

为具有不全外显率的常染色体显性遗传病。目前认为是mRNA的不稳定,转录后控制机制缺陷所致。

4、性联隐性鱼鳞病:

为X染色体连锁隐形遗传。类固醇硫酸酯酶基因(STS)缺失或突变,造成硫酸胆固醇积聚,角质层细胞结合紧密不能正常脱落,形成鳞屑。

先天性非大疱性鱼鳞病样红皮病:

为常染色体隐性遗传,由多个基因如脂氧合酶12(R)(ALOX12B)基因、油脂氧化酶3(ALOXE3)基因的突变引起。

5、迂回性线状鱼鳞病:

为常染色体隐性遗传病,是由染色体5p32上的丝氨酸蛋白酶抑制剂Kazal5型(SPINK5)基因突变造成。

鱼鳞病的症状

主要表现为四肢伸侧或躯干部皮肤干燥、粗糙,伴有菱形或多角形鳞屑,外观如鱼鳞状或蛇皮状,重者皮肤皲裂、表皮僵硬、导致自身汗毛稀少、排汗异常,致使体内水液代谢失衡,影响内分泌系统。

组织病理改变:

1、显性遗传性鱼鳞病:表皮中度角化过度,伴颗粒层变薄或消失;

2、性联寻常型鱼鳞病:角化过度,颗粒层正常或稍厚;

3、表皮松解角化过度鱼鳞病:角化过度,表皮细胞松解,颗粒变性。

鱼鳞病西医治疗

一、鱼鳞病全身治疗:

可试用较大剂量维生素A注射或内服,也可应用芳香维生素A酸乙酯、维生素E等治疗。

二、鱼鳞病局部治疗:

1、外用润肤的油膏,如10%尿素软膏、0.1%维生素A酸软膏及30%鱼肝油软膏等;2、局部应用角质松解剂,如5%水杨酸软膏、5%硫黄煤焦油软膏或40%—60%丙二醇水溶液等;3、可酌情选择紫外线照射、淀粉浴或矿泉水浴等。

三、鱼鳞病药物治疗方法:

1、内服法:口服大剂量维生素A,每日10~30万u或维生素A酸,每日1mg/kg/日。但长期服用可出现骨质脱钙,脱发和其他中毒症状。

2、外治法:外用10%尿素脂,0.1%维生素A酸霜或20%鱼肝油软膏等,对缓和皮肤干燥、脱屑、皲裂有一定疗效。

鱼鳞病中医治疗

1、中成药:①十全大补丸,适用于血虚风燥证患者;②大黄廑虫丸,适用于瘀血阻滞证患者。

2、辨证施治:①血虚风燥证,治以养血活血、润燥熄风,方用养血润肤饮加减;②瘀血阻滞证,治以活血化瘀、养肤润燥,方用血府逐瘀汤加减。

3、外治疗法:①皮肤干燥、粗糙、脱屑,可选用杏仁、桃仁、胡麻仁、郁李仁、火麻仁及胡桃仁各适量,共捣烂如泥,水煎后外洗,外涂胡桃膏或羊髓膏;② 皮肤粗糙、增厚、皲裂、可选用大黄、透骨草、桂枝、桃仁、当归、丹参、地骨皮、皂角刺,煎水外洗,然后外涂润肌膏或当归膏。

鱼鳞病能治好吗

1、目前国内外还没有治愈好鱼鳞病的方法,因此我们并不能从根治愈好鱼鳞病,我们只能最大程度的降低鱼鳞病的遗传几率,延长鱼鳞病的复发时间。不过,患上这种病,患者本身一般没有明显的异常感,只是在冬季由于汗腺及皮脂腺分泌减少,皮肤干燥会感到瘙痒不适,如果出现皮肤皲裂,可能还会感到疼痛,但一般对患者的健康危害不大,对其寿命不会有影响,只是对外观和心理会造成影响。

2、但有研究表明,鱼鳞病患者血清中维生素A的含量明显低于正常人,他们的机体对维生素A的利用有先天性缺陷。因此,建议患有此类病的患者日常可适当补充维生素A,比如,可口服或肌肉注射维生素A,维生素A为脂溶性维生素,一般可以在饭后服,但要注意别服用过量。还可外用尿素霜、鱼肝油软膏、∝-羟基酸、50%的丙二醇溶液等增加皮肤水合程度,用维A酸外用制剂或钙铂三软膏等可改善角化程度,减少鳞屑。

3、而在日常护理上,平时可多吃富含维生素A的食物,如胡萝卜、白薯、绿叶菜及猪肝等。另外,冬季洗澡不宜过勤,洗时最好不用肥皂,洗澡后要搽护肤油脂,可使皮肤变得柔润,鳞屑减少,并保持适当的水分和足够的营养成分。除此之外,还应注意保暖,避免风寒刺激皮肤,忌食辛辣刺激食物。

4、特别需要提醒的是,溶血性链球菌感染是本病的一大诱发因素,应尽可能避免感冒、扁桃体炎、咽炎的发生。一旦发生感染应积极对症治疗,以免加重病情。 

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