中华皮肤科杂志 ›› 1994, Vol. 27 ›› Issue (4): 203-205,268.

• 论著 •    下一篇

重型种痘样水疱病的临床研究

顾恒1, 钱恒林1, 常宝珠1, 李光中2   

  1. 1. 中国医学科学院、中国协和医科大学皮肤病研究所理疗科, 南京, 210042;
    2. 指导者
  • 收稿日期:1993-10-26 修回日期:1994-01-20 出版日期:1994-08-15 发布日期:1994-08-15

Clinical Study on Severe Hydroa Vacciniforme

GU Heng1, QIAN Heng-Lin1, CHANG Bao-Zhu1   

  1. Institute of Dermatology, Chinese Academy of Medical Sciences, Peking Union Medical College, Nanjing 210042
  • Received:1993-10-26 Revised:1994-01-20 Online:1994-08-15 Published:1994-08-15

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摘要： 9例重型种痘样水疱病患者均为日晒后暴露部位出现红斑、丘疹、水疱、糜烂、溃疡等,反复发作,渐致畸形.5例有手部畸形,表现为1、2、3指关节强直或屈曲、错位;2例指骨部分吸收破坏;5例耳廓部分缺损;2例鼻梁塌陷,软骨部分破坏吸收;1例下唇瘢痕挛缩,门齿外露;4例有角膜混浊.实验室检查均可排除卟啉代谢障碍性疾病,光试验对UVA反应异常.因患者均有不同部位的畸形,故称为重型种痘样水疤病,并认为夏令痒疹、夏令水疱病、种痘样水疱病、重型种痘样水疱病是由光所致的谱性疾病.

关键词: 牛痘样水疱

Abstract: Nine cases of severe hydroa vacciniforme are reported.All the cases had erythema, papules, bullae, erosions or ulcers on exposed sites, with recurrences and gradually resulted in deformation.Five patients suffered from hand deformation manifesting rigidity, flexor tetanus or malposition of the first, second and third interphalangeal joints of hands.Two of the above mentioned patients manifested partial bone absorption of fingers.Five patients showed partial defect of auricle.Two patients showed saddle nose, and partial absorption of nasal cartilage.One patient displayed cica trical contracture of lower lip, with incisor extrusion.Four patients revealed opacity of cornea.Through laboratory examinations, porphyrinopathies were excluded.Phototest showed increased sensitivity to UVA.Based on the clinical features and laboratory examinations,these patients were diagnosed as severe hydroa vacciniforme.It is believed that summer prurigo, hydroa aestivale, hydroa vacciniforme and severe hydroa vacciniforme all belong to a disease spectrum induced by UV light.

Key words: Hydroa vacciniforme