Weber B P, Fierlbeck G, Kempf H G
Department of Otorhinolaryngology, Medizinische Hochschule Hannover, Germany.
Eur Arch Otorhinolaryngol. 1994;251(6):342-6. doi: 10.1007/BF00171541.
Data from a young adult man with epidermodysplasia verruciformis (EV) and multiple metachronous spinaliomas in the head and neck region are presented. Diagnosis of this rare, human papilloma virus (HPV)-associated disease was based on: (1) Typical skin lesions, including viral warts, verruca plana-type lesions and pityriasis versicilor; (2) typical histological features, including "foamy giant keratinocytes"; (3) evidence of HPV 5, 8 and 20 in pityriasis versicilor-like lesions; (4) a cellular immunodeficiency due to a relative T-helper-cell deficit. No specific treatment of EV is known, so that therapy concentrates on early removal of spinaliomas and treatment of intercurrent infections. Since EV patients have numerous benign skin lesions and frequently develop metastatic and non-metastatic carcinomas, molecular changes of HPV during carcinogenesis can be studied.
本文报告了一名患有疣状表皮发育不良（EV）且头颈部出现多个异时性脊髓瘤的年轻男性患者的数据。这种罕见的、与人乳头瘤病毒（HPV）相关疾病的诊断基于：（1）典型的皮肤病变，包括病毒疣、扁平疣样病变和花斑癣样病变；（2）典型的组织学特征，包括“泡沫状巨大角质形成细胞”；（3）花斑癣样病变中HPV 5、8和20的证据；（4）由于相对的辅助性T细胞缺陷导致的细胞免疫缺陷。目前尚无已知的EV特异性治疗方法，因此治疗集中于早期切除脊髓瘤和治疗并发感染。由于EV患者有大量良性皮肤病变，且经常发生转移性和非转移性癌，因此可以研究HPV在致癌过程中的分子变化。
