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本病又称Hutchinson—Gilford氏综合症。是一种先天性遗传性疾病。但还不能确定是常染色体隐性还是显性遗传。特点为发育迟延,至婴儿时期就发生进行性老年性退行性改变,具有老人貌,但智力发育尚可。本文报告两例,是姐弟两,例1女性16岁。例2男性,12岁。都是从八岁时发病,先是从面部开始出现散在的紫红色小丘疹、以后皮肤萎缩留下瘢痕色素沉着,这一过程反复发作,逐渐侵犯全身皮肤和粘膜,口腔、咽喉部粘膜为重,出现吞咽困难声音嘶哑。
This disease is also called Hutchinson-Gilford’s syndrome. Is a congenital genetic disease. But it is still not certain whether autosomal recessive or dominant inheritance. Characterized by the developmental delay, to the occurrence of progressive degenerative senile deformities in infancy, with the elderly appearance, but mental development is acceptable. This article reports two cases, two siblings, one female 16 years old. Example 2 male, 12 years old. Are from the age of eight onset, first from the face began to appear scattered purple papules, after the skin atrophy left scar pigmentation, the process repeated attacks, and gradually violations of the whole body skin and mucous membranes, mouth, throat mucosa, Hissing dysphagia occurs.