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先先天性皮肤发育不全(ACC)有罕见的先天性局部皮肤的缺失,多位于中线或近中线的头皮,病变常较浅表。某些病区较广泛。可累及深层皮肤、下面的骨骼甚至内部组织。原发性道淋巴管扩张(IL)是另一罕见疾病。其全身性或局限性淋巴管病变引起许多异常。包括失蛋白性肠病、低蛋白血症、水肿和淋巴细胞减少。此两病都可散发或家族性发病。本文报告1家两例并存这两种疾病,提示控制中、外胚层发育基因有遗传性缺陷。病例例1 男,3岁半。出生时,头皮有1×2 cm卵形无发疤痕区,其下骨质缺损。眼眶周围水肿、下肢不可凹陷性水肿、左掌有猿纹。生后6个月水肿自然消失。25岁时发现近视眼和视乳头两侧脉络膜裂。3岁时又出现全身性水肿。头顶疤痕区扩
First of congenital hypoplasia (ACC) there is a rare congenital absence of local skin, mostly in the midline or near the midline of the scalp, lesions often superficial. Some wards are more extensive. Can affect the deep skin, the underlying bone or even internal tissue. Primary vaginal dilatation (IL) is another rare disease. Its systemic or localized lymphangitic lesions cause many abnormalities. Including loss of egg white enteropathy, hypoproteinemia, edema and lymphopenia. Both diseases can be distributed or familial disease. This article reports a coexistence of two patients with one of two diseases, suggesting that the control of ectodermal development of genetic defects. Case 1 male, 3 years and a half. At birth, the scalp has a 1 × 2 cm oval hairless scar area, the underlying bone defects. Periorbital edema, lower limbs can not be depressed edema, left palm with ape pattern. 6 months after birth, edema naturally disappear. 25 years old, myopia and optic chiasm were found on both sides of the choroid. 3-year-old systemic edema again. Head scar area expansion