回旋形线状鱼鳞病一家系调查

A pedigree of ichthyosis linearis circumflexa

报告1例回旋形线状鱼鳞病患者的家系调查,该家系3代14人中仅有母女2人患病。2例患者均表现为出生后不久全身出现泛发性环形红斑和鳞屑,掌跖角化过度,无毛发异常。组织病理学检查显示:表皮轻度疣状增生,颗粒层变薄,棘层肥厚,角化过度等非特异性改变。

A pedigree of ichthyosis lincaris circumflexa is reported.There were two family members of three genrations affected.Two patients had clinical features of widespread circinated erythema and scale,with hyperkeratosis of palms and sales.There skin lesions developed soon after birth.Evidence of hair shaft abnormatlities could not be found.Histopathological examination showed non-specific changes of acanthosis and hyperkeratosis.