三种鱼鳞病的病理及超微结构对比研究

Study on pathological and ultrastructural comparison of three types of ichthyosis

目的:对寻常型鱼鳞病(ichthyosis vulgaris,IV)、板层状鱼鳞病(lamellar ichthyosis,LI)和大疱性先天性鱼鳞病样红皮病(Bullous congenital ichthyosiform erythroderma,BCIE)患者进行临床及病理超微结构分析,归纳总结其特点。方法:随访并收集12例寻常型鱼鳞病、板层状鱼鳞病和大疱性鱼鳞病患者的临床资料及皮损,对比三种鱼鳞病患者皮损病理及超微结构的特点及改变。结果:诊断IV5例、LI4例,BCIE3例,IV组织病理学表现轻度角化过度及棘层轻度增生,超微结构显示角质层层数增多,角蛋白密度均匀,角质化包膜连续,脂质颗粒较少;LI组织病理学示高度黏附的角化过度及增生的颗粒层、棘层,超微结构示角质层层数增多、角蛋白密度不均,角质化包膜连续,较多脂质颗粒;BCIE组织病理学示高度角化过度及角化不全,颗粒层增厚且透明角质颗粒异常增多、凝结,棘层轻度增生,超微结构示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围绕异常聚集的角蛋白微丝。结论:电镜超微病理观察对鱼鳞病分类有辅助诊断价值。

Objective To analyze the clinical and pathological changes of the structure of ichthyosis vulgaris, lamellar ichthy-osis and ichthyosis bullosa of patients, summarize its characteristics. Method 12 cases of ichthyosis vulgaris, lamellar ichthyosis and ichthyosis bullosa of patients with clinical data were collected in my Department of Dermatology and comparison of three kinds of skin lesions, skin lesions of patients with ichthyosis clinical characteristics, pathological and ultrastructural chan-ges. Results The diagnosis of IV 5 cases,LI 4 cases, BCIE 3 cases showed mild hyperkeratosis and acanthosis hyperplasia IV his- topathology, ultrastructure showed cuticle layer increased, keratin uniform density, cornified envelope, less lipid particles ; particle layer, spinous layer LI histopathology showed hyperkeratosis and high adhesion the ultrastructure showed hyperplasia,stratum cor- neum keratin layer increased, uneven density, continuous cornified envelope, a lot of lipid particles ; BCIE histopathology showed high hyperkeratosis and parakeratosis, hypergranulosis and keratohyalinegranules abnormal increase, condensation, spinous layermild hyperplasia, ultrastructure showed keratin filament structure disorder a tear shaped, granular layer and upper spinous layer a-round the nucleus around the abnormal accumulation of keratin filaments. Conclusion The diagnosis of ichthyosis may have diag-nostic value in electron microscope pathological observation.