摘要
目的分析家族性大疱性扁平苔藓(BLP)的临床和遗传特点。方法5个家族性BLP的先证者经临床和病理诊断后,对其家庭成员进行体检,收集临床和遗传资料。结果5个家族性BLP中共有成员52人,男27人,女25人,其中BLP患者24人,平均患病率为46.2%。男性患者9例(33.3%),女性患者15例(60.0%),女性患病率明显高于男性,χ2=16.4,P<0.01。皮疹主要分布于下肢胫前,其次为上肢和大腿。83.3%的患者尼氏征阳性,同形反应阳性率为62.5%。患者皮疹特别是水疱或大疱常于夏季加重,而冬季减轻。在同一家族患者中,病情为父重女轻、母重女轻、兄重弟轻、姐重妹轻。发病年龄越小,指甲越有可能受累。结论家族性BLP有其自身特殊的临床特点。
Objective To analyze the clinical and inheritance features of familial bullous lichen planus （BLP）. Methods Five probands of BLP were diagnosed by clinical onsets and histopathology. Then the clinical and inheritance data for all the family members of these 5 pedigrees were collected. Results In a total of 52 family members of these 5 pedigrees there were 24 BLP patients with 9 males and 15 females. The mean morbidity rate was 46.2%. The morbidity rate for male and female was 33.3% and 60.0% respectively, χ^2 = 16.4, P〈0.01. The skin lesions had a predilection on both shines. The next predilection areas were upper limbs and thighs. Nikolsky＇s signs and isomorphism reaction were found positive in 83.3% and 62.5% patients respectively. Skin lesions especially vesicles and bulla became worse during summer and ameliorated during winter. The severity of the disease in one family showed father was severer than daughter, mother was severer than daughter, elder brother was severer than younger one and elder sister was severer than younger sister. The earlier did the patient develop the disease the more possible the nails would be involved. Conclusion Familial BLP had its own special clinical features.
出处
《华中科技大学学报（医学版）》
CAS
CSCD
北大核心
2007年第3期377-379,共3页
Acta Medicinae Universitatis Scientiae et Technologiae Huazhong
