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本文报告1家两例先天性皮肤发育不全(ACC)和原发性肠道淋巴管扩张(IL)并存。例1,男,3岁半。出生时头皮有1×2cm卵形无发瘢痕区,其下骨质缺损。眶围水肿,下肢非凹陷性水肿,左掌猿纹。生后6个月水肿自然消失。2岁半时发现近视和两侧视乳头缺损。3岁时又出现全身水肿。头顶瘢痕区扩大到5×6cm。实验室检查:白细胞4,500,淋巴细胞800。血桨总蛋白2.7g/dl(白蛋白1.7g/dl,球蛋白1.0g/dl)。IgG 180mg/dl,IgA43mg/dl,IgM47mg/dl,血钙7.6mg/dl,血磷5.2mg/dl。尿量150～250ml/d,尿常规正常,尿培养(-)。头颅片正常。钡餐:小肠普遍水肿,粘
This article reports a coexistence of two cases of congenital hypoplasia (ACC) and primary intestinal lymphatic distension (IL). Example 1, male, 3 and a half years old. The scalp at birth is 1 × 2cm oval without scarring area, the underlying bone defects. Orbital edema, non-pit lower edema, left ape pattern. 6 months after birth, edema naturally disappear. 2 years and a half found myopia and bilateral optic nerve defect. 3-year-old body edema again. Head scar area expanded to 5 × 6cm. Laboratory tests: 4,500 leukocytes, 800 lymphocytes. Total plasma protein 2.7g / dl (albumin 1.7g / dl, globulin 1.0g / dl). IgG 180mg / dl, IgA 43mg / dl, IgM47mg / dl, calcium 7.6mg / dl, phosphorus phosphorus 5.2mg / dl. Urine output 150 ~ 250ml / d, urine normal, urine culture (-). Headshot normal. Barium meal: the general edema of the small intestine, sticky