遗传性口形细胞增多症是一种罕见的常染色体疾病,国外截至1979年共报告43例,国内1984年迄今仅见4例报道本文报告2例,情况如下: 例1:女,13岁,回族,于1978年5月15日入院。自述近三年多来反复出现巩膜及皮肤黄染,有时发生鼻衄及牙龈出血。病程中无寒战发热或消化功能紊乱等表现。否认曾有肝炎病史或服用过某些可致溶血、肝损害药物或食物史。其兄亦曾有贫血及巩膜黄染史。体检:发育较同龄人矮小,营养稍差,皮肤及 Hereditary oral bullous disease is a rare autosomal disease reported in foreign countries as of 1979, a total of 43 cases reported in China in 1984 so far only 4 cases reported in this report 2 cases, the situation is as follows: Example 1: Female, 13 years old, Hui, In May 15, 1978 admission. Read more than three years since the recurrence of sclera and skin yellow dye, epistaxis and gingival bleeding sometimes occur. No chills in the course of fever or digestive disorders and other manifestations. Denied having had a history of hepatitis or had taken some can cause hemolysis, liver damage drugs or food history. His brother also had history of anemia and sclera yellow dye. Physical examination: development shorter than their peers, less nutritional, skin and