推荐回答(1个)
线状表皮痣(linear epidermal nevi),又称疣状痣(verrucous nevi).是一种先天性限局性表皮发育异常,表皮呈疣状增生为特点的表皮肿瘤.
【临床表现】
(一)限局型线状表皮痣常见于出生时,为单个线状皮损.由密集的乳头瘤样,过度角化性丘疹,连续或间断排列成条状隆起皮损.呈正常肤色,淡红,黄褐色至黑色,境界清楚,发生于头部,躯干或四肢的任何部位,一般只发生于身体的一侧,因此又称单侧痣(nevus unius fateris).如发生于四肢多呈纵行线状或螺旋形排列;发生于躯干者呈横行带状或波纹状排列,但多不超过中线.皱襞部损害可浸渍发白,易受感染而产生臭味.侵犯指趾时,可致甲显著扭曲畸形和反复发生甲沟感染.粘膜亦可受累呈乳头状突起.
(二)系统型线状表皮痣与限局型线状表皮痣的线状皮损相同,不同之处是线状皮损不只一条,而是许多条皮损平行排列,特别是位于躯干者.皮损位于一侧,偶亦呈双侧对称分布.
限局性线状表皮痣尤其是系统性线状表皮痣,均可伴发骨骼和神经系统疾患,如智力发育迟缓,癫痫和神经性耳聋.偶亦可在皮损上继发基底细胞上皮瘤和鳞状细胞癌.
【病理说明】
限局性线状表皮痣和某些系统性线状表皮痣呈现中等度角化过度,乳头瘤样增主,棘层肥厚和表皮突延长.表皮松解性角化过度(顺粒层和棘层细胞核周空泡化,空泡化周围细胞边界不规则,大而不规则的透明角质颗粒增多,角化过度,角质层致密)可见于系统型线状表皮痣,偶见于限局型线状表皮痣.
【鉴别诊断】
幼年发病呈线状疣状隆起性皮损,诊断不难,但应与一些线状皮损的疾病鉴别.
1.线状苔藓(1ichen striatus)为炎症性线状皮炎,发展迅速,具有自限性,大多在3一6个月消退,遗留暂时性色素脱失斑.组织病理无特异性,表皮可见细胞内及细胞间水肿,无棘层肥厚,真皮血管周围有中等度慢性炎症性浸润.
2.炎症性线状疣状表皮痣(inflammatory linear verrucoas epidermal nevus)临床上呈红斑瘙痒性炎性皮损.组织学上出现角化不全,真皮慢性炎症浸润,可以鉴别.
3.线状汗孔角化病(linear porokeratosis)周缘往往有堤状角化性隆起,组织病理可见特征性角化不全柱(鸡眼样板cornoid 1amel1a),其下颗粒层消失.
4.线状扁平苔藓(lichen planus linearis〕由紫红色多角形扁平丘疹,簇集呈带状排列,自觉剧痒,组织病理检查,具有特征性.
5.线状银屑病(1inear psOriasis)皮损虽呈线状,但仍具寻常型银屑病的临床特点,组织病理可以鉴别.
应在青春期皮损停止扩展后进行.可行手术切除植皮,刮术,化学剥皮法(三氯醋酸或苯酚外用),激光或冷冻疗法.亦可试用维A酸软膏,水杨酸软膏或5-氟尿嘧啶软膏外用姑息治疗.
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