报告了28例系统性硬皮病(PSS),包括三个家系的HLA表型。其中女26例,男2例,发病年龄39±12岁,就诊年龄53±13岁,病期14±9年(1～37年)。并以4例MCTD和4例泛发性硬斑病作对照。三个家系病例报告如下:例1-1.女,58岁,关节痛10年,雷诺现象和皮肤硬化1年,伴食道功能低下3月,因呼吸功能不全死亡;例1-2.51岁,为例1-1的胞妹,雷诺现象,面和手部硬化3年。间接免疫荧光ANA阳性,呈斑点型和核型。例2-1.女,52岁, Reported 28 cases of systemic scleroderma (PSS), including HLA phenotypes in three families. Among them, 26 were women and 2 were males. The age of onset was 39 ± 12 years. The age of treatment was 53 ± 13 years and the duration of illness was 14 ± 9 years (range 1 to 37 years). And 4 cases of MCTD and 4 cases of urticarial sclerosis as a control. Cases of three pedigrees are reported as follows: Example 1-1 Female, 58 years old, arthralgia for 10 years, Raynaud’s phenomenon and sclerosis for 1 year, with esophageal dysfunction March, death due to respiratory insufficiency; cases 1-2.51 years old, Example 1-1, sister, Reynolds phenomenon, facial and hand sclerosis for 3 years. Indirect immunofluorescence ANA positive spots and karyotype. Example 2-1. Female, 52 years old,