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患儿,男,3天。1988年2月30日入院。第一胎,足月顺产。出生时全身皮肤蒙一层黄色包膜,紧而发亮伴轻度水肿。父母非近亲结婚,母妊娠期身体健康,未用任何药物。家族中无类似病史。体检:肛温37.8℃,脉搏140次/分,呼吸38次/分,体重3100g。全身皮肤犹如一层干燥的火棉胶样薄膜,发亮,四肢屈曲,伸展时皮肤扯紧。眼角膜干燥,结膜充血。面部皮肤紧张呈张口状,吞咽功能尚好。心肺未见异常。手足如蜡状、水肿、僵硬,颈、肘、腹股沟处皮肤有条状皲裂,少许渗液(见图1*)。外周血白细胞17.2×10~9/L、N O.58、L 0.40、M 0.02。皮肤病理检查:毛囊口角化,表皮
Children, male, 3 days. February 30, 1988 admission. The first child, full-term follow-up. Whole body skin at birth was covered with a yellow envelope, tight and shiny with mild edema. Parents non-relatives get married, mother’s health during pregnancy, did not use any drugs. No similar family history. Physical examination: rectal temperature 37.8 ℃, pulse 140 beats / min, breathing 38 beats / min, weight 3100g. Whole body skin is like a layer of dry fire cotton glue-like film, shiny, limb flexion, stretched skin tight. Cornea dry, conjunctival hyperemia. Facial skin tension was mouth-swollen, swallowing function is still good. Heart and lung no abnormalities. Hands and feet, such as waxy, edematous, stiff, neck, elbow, groin skin chapped, a little exudate (see Figure 1 *). Peripheral blood leukocytes 17.2 × 10 ~ 9 / L, N O.58, L 0.40, M 0.02. Skin pathology: hair follicles mouth, epidermis