疣状表皮发育不良2例

例1.男,25岁.因全身泛发淡红色丘疹15年,于2007年3月25日来我科就诊.患者于15年前不明原因双手背出现数十个绿豆大淡红色和浅褐色扁平丘疹,无自觉症状,未诊治.以后皮损逐渐增多、扩展,面、颈部及躯干部也出现类似皮损.曾在当地医院诊断为扁平疣、扁平苔藓,予外用糖皮质激素、维A酸软膏治疗,皮损未明显消退.     

疣状表皮发育不良1例报告

疣状表皮发育不良又称泛发性疣病,为一种少见的泛发性、多形性由人乳头瘤病毒感染所致的疣皮肤病.现将我们发现的1例具有两种皮损表现的病例报告如下.临床资料患者,男,17岁,学生,莒县人.双手背、面、颈部扁平疣状皮疹12年.患者于12年前不明原因的在双手背开始出现暗红色扁平疣状丘疹,无自觉症状,未在意.     

疣状表皮发育不良

疣状表皮发育不良(epidermodysplasin verruciformis,EV)是由人类乳头瘤病毒(HPV)引起,以表皮细胞空泡变性为特征,易发生恶变,常有遗传背景的皮肤病。由于EV相关的HPV病毒与一般人群的皮肤及生殖道等癌肿密切相关而引起关注^[1]。近年来,随着流行病学、病毒学及临床的深入研究,对EV有了更多新的认识。     

疣状表皮发育不良并发巨大尖锐湿疣1例

疣状表皮发育不良并发巨大尖锐湿疣１例韩科，蒙秉新，杨海鹰患者女，１８岁，学生，就诊于１９９２年９月１３日。于９岁时双手手指伸侧、手背等处出现少许米粒大淡红或浅褐色皮疹。初起时扁平隆起，轻度瘙痒，继而部分融合，表面呈疣状，同时向四肢、躯干及外阴部扩展，...     

阿维A治疗疣状表皮发育不良1例

疣状表皮发育不良是由人类乳头瘤病毒（HPV）感染导致的与遗传有关的少见皮肤病,临床上可分为扁平疣型、花斑癣型及点状瘢痕型。花斑癣型疣状表皮发育不良较少见,临床上表现为泛发性花斑癣样皮损,治疗较困难。笔者采用阿维A治疗1例泛发性花斑癣型疣状表皮发育不良,取得较好疗效,现报告如下。[第一段]     

点状瘢痕型疣状表皮发育不良1例

报告1例以少见的点状瘢痕为表现的疣状表皮发育不良。患者男,17岁。面颈、躯干、上肢大量点状轻微凹陷的斑疹12年。皮损组织病理示:表皮中上部有明显的弥漫性细胞空泡化。诊断:疣状表皮发育不良(点状瘢痕型)。予重组人α-2b干扰素凝胶外搽治疗。     

阿维A治疗疣状表皮发育不良1例

报告1例疣状表皮发育不良。患者男,57岁。下腹、前臂、会阴和大腿近端出现乳头瘤样增生物7年,躯干部泛发扁平疣样皮损,手、足部出现寻常疣样皮损5年。给予小剂量阿维A治疗6个月取得满意疗效,随访7个月皮损无复发。     

花斑癣型疣状表皮发育不良1例

花斑癣型疣状表皮发育不良１例陈昭渭，刘迎红（门诊部）刘榕城，（病理室）（福建省福州市皮肤病院，３５０００１）花斑癣型疣状表皮发育不良（ＰｉｔｙｒｉａｓｉｓＶｅｒｓｉｃｏｌｏｕｒ－ｌｉｋｅｅｐｉｄｅｒｍｏｄｙｓｐｌａｓｉａＶｅｒｒｕｃｉｆｏｒｍｉｓ）较...     

疣状表皮发育不良1例

1　临床资料患儿男 ,15岁。因双手背、前臂、双足背及小腿丘疹10年 ,于 2 0 0 2年 1月 18日就诊。 1992年 8月患儿因病毒性脑炎出现嗜睡状 ,发烧 ,伴口唇疱疹 ,在本地医院治疗 ,约2周后意识障碍恢复 ,体温正常 ,疱疹消退 ,但遗留继发性癫痫和一定的智力减退。后双手背、足背出现米粒至黄豆大的扁平丘疹 ,自觉轻微瘙痒 ,常搔抓皮疹 ,未诊治。随年龄的增长 ,皮疹逐渐增多 ,且蔓延至双前臂、双小腿 ,曾求治于多家医院及个体诊所 ,治疗无好转 ,皮疹数目增多 ,伴双手背及前臂皮肤增厚 ,至今经久不退而就诊于我院。否认家族中有类似疾病病史。体格…     

疣状表皮发育不良并发鳞状细胞癌1例

1 病历摘要 患者男,34岁.因全身丘疹30年伴额部斑块1年余,于2011年5月21日至我院就诊.患者于30年前无明显诱因,全身出现散在的红色斑丘疹,并逐渐扩大,不能自行消退.近一年来额面部出现结节,并逐渐增大,破溃结痂不能愈合.患者未正规就诊及治疗,外用各种药物(具体不详)治疗效果不佳.有乙肝病史,职业为农民,经常日晒.父母非近亲结婚,患者系足月顺产,家族中无类似疾病.     

Acquired epidermodysplasia verruciformis in a renal transplant recipient - Case report

A 24-year-old male patient, who underwent kidney transplant six years ago due toLupus nephritis, for the last two years presented asymptomatic erythematous scalyplaques on the abdomen and areas exposed to light. Post-transplantationimmunosuppressive medications included prednisone, mycophenolate sodium andsirolimus. The histopathologic features were typical for epidermodysplasiaverruciformis. Epidermodysplasia verruciformis is a rare autosomal recessivegenodermatosis with increased susceptibility to specific strains of cutaneous humanpapilloma virus. The term ''''acquired epidermodysplasia verruciformis'''' was recentlyintroduced to the literature and describes epidermodysplasia verruciformis occurringin patients with impaired cell-mediated immunity. We report an additional caseassociated to immunosuppression after kidney transplantation.     

疣状表皮发育不良患者皮损中IL-10、IL-23和CD86的表达

目的 观察疣状表皮发育不良（EV）患者皮损中细胞因子IL-10、IL-23和CD86的表达情况,探讨细胞免疫异常与EV发病的关系。方法 采用免疫组织化学方法检测10例EV患者皮损细胞因子的表达,并以10例正常人的眼皮标本作为对照。结果 EV组中三种细胞因子表达为阳性,而正常组有1例IL-10阳性,其余均为阴性;EV组中三种细胞因子的评分为3～6分,而正常组除1例IL-10评分为3分,其余均为0分;EV组中三种细胞因子的阳性表达强度为（++）～（+++）,而正常组中除1例IL-10为（++）,其余均为（-）。IL-10, IL-23, CD86三种细胞因子在EV组和对照组间阳性表达率有明显差异。结论疣状表皮发育不良的发病可能和角质形成细胞分泌的细胞因子异常有关。     

Seborrheic Keratosis-like lesions in patients with epidermodysplasia verruciformis

Epidermodysplasia verruciformis (EV) is a rare genodermatosis characterized by disseminated infection by human papillomavirus (HPV) and malignant transformation of the lesions in about half of the patients. Two phenotypes of EV have been described according to their propensity to develop malignant tumors. The benign form of EV presents a singular type of lesions comprised of flat warts widely disseminated. The malignant form of EV is highly polymorphic and presents as malignant skin tumors, predominantly basal and squamous cell carcinomas, on sun-exposed sites. The seborrheic keratosis-like (SK) lesions in patients of EV have been reported to be associated with the malignant phenotype. In this work, we documented the behavior of SK-like lesions in nine patients with EV, through clinical observations as well as histological and immunohistochemical findings. We suggest that the HPV infection may promote the occurrence of SK-like lesions in EV patients. Despite the fact that we did not observe any malignant transformation of these lesions in our series of patients, this possibility was not completely excluded.     

HPV43／66感染致疣状表皮发育不良一例

患者男,37岁。因全身多发疣状丘疹、斑片20年,外阴和腹股沟糜烂、渗出2个月就诊。皮肤科检查：全身多发、蚕豆大疣状丘疹,部分融合成片。双侧腹股沟及外阴皮损处糜烂、渗液和结痂。外周血淋巴细胞亚群分析示：CD4＋T细胞37．2％,CD8＋T细胞43．6％,CD4＋／CD8＋细胞比值0．85。斑点杂交示：HPV43和66DNA阳性。皮损组织病理：角化过度,表皮疣状增生及假上皮瘤样增生,可见部分凹空细胞,真皮浅层中度淋巴细胞浸润。诊断为疣状表皮发育不良、皮肤感染和细胞免疫功能低下。治疗给予抗感染、肌内注射免疫增强剂和口服阿维A胶囊,治疗15d后外阴和腹股沟皮损表面干燥、变平,并有大量痂屑脱落,而躯干和四肢皮损无明显变化。目前患者仍在随访中。     

Combined treatment of epidermodysplasia verruciformis with etretinate and α-interferon*

Epidermodysplasia verruciformis (EV) is an uncommon cutaneous disease in which a focal and genetically determined immunological impairment is associated with chronic human papilloma virus (HPV) infection. In sun-exposed areas, when an oncogenic HPV type is the agent, skin cancer may occur. The treatment of EV is difficult and often unsatisfactory; etretinate has been reported in some instances as effective in improving lesions. We report a typical case of EV with pityriasis versicolor-like lesions on the trunk and many flat, erythematous wart-like lesions on the face, dorsal areas of the hands and legs. We performed a treatment with etretinate (1 mg/kg/day for 6 weeks) and subsequently with etretinate and α₂r-interferon (3 MU, 3 times per week for 2 weeks, then 6 MU, as above, for 4 weeks). After 4 weeks of therapy with etretinate we observed moderate improvement; we did not observe any further clinical improvement in the final 2 weeks. The subsequent combined treatment with etretinate and a-interferon achieved further improvement. We conclude that the association of etretinate and a-interferon may represent an efficacious treatment of EV.     

Benign and malignant hybrid adnexal tumors in a patient with epidermodysplasia verruciformis

Epidermodysplasia verruciformis (EV) is a genodermatosis characterized by overgrowth of flat warts, pityriasis versicolor‐like lesions and an increased propensity for developing cutaneous squamous cell carcinomas due to abnormal susceptibility to infection with beta‐human papilloma viruses. Adnexal tumors are not typically associated with EV. Here we report a spectrum of hybrid adnexal tumors with divergent eccrine and folliculosebaceous differentiation, and cytologic features ranging from benign to frankly atypical, in a patient with inherited EV.     

Clinical aspects of epidermodysplasia verruciformis

Thirteen patients with epidermodysplasia verruciformis (EV) were studied over a period of 7 years. EV is a rare genodermatosis characterized by a generalized infection with a specific group of human papilloma virus (HPV) and a propensity for developing skin malignant tumours in 30%-50% of patients. The diagnosis of EV was confirmed by histopathological and immunohistochemical findings. Three of our patients had the benign form of EV, which is characterized by monomorphous lesions and no malignant changes, whereas 10 had the malignant form, which is characterized by polymorphic lesions and development of cutaneous malignant tumours. All EV patients with the malignant form developed multiple skin tumours (77%). They started to appear at age 20, predominantly on the forehead (50%). Most were squamous cell carcinoma, extremely aggressive and invasive, which provoked metastasis and death in two patients.     

Distinctive distribution of human papillomavirus type 16 and type 20 DNA in the tonsillar and the skin carcinomas of a patient with epidermodysplasia verruciformis

BACKGROUND: Epidermodysplasia verruciformis (EV) is a rare skin disease characterized by disseminated pityriasis versicolor-like or flat wart-like lesions and by the development of skin carcinomas. It is well established that specific cutaneous human papillomaviruses (EV-HPVs) are associated with both benign and malignant skin lesions in EV patients. However, little is known of the relationship between HPV and the mucosal lesions of EV patients. OBJECTIVES: To detect and identify HPV types associated with skin and mucosal lesions of an EV patient. PATIENT/METHODS: We investigated the skin carcinoma and the coexisting tonsillar carcinoma of a 41-year-old man with EV. Histopathologically, both lesions were squamous cell carcinomas. We analysed these two lesions by immunohistochemistry, in situ hybridization, and by molecular virology. RESULTS: Neither skin nor tonsillar lesions exhibited positivity for HPV capsid antigen by immunohistochemistry. By Southern blot hybridization, however, the skin carcinoma harboured 'EV-specific' HPV20 DNA, while the tonsillar carcinoma harboured 'genital' HPV16 DNA. In addition, in situ hybridization localized the respective viral DNA in the corresponding lesion. CONCLUSIONS: The results indicate that EV-HPV could be responsible for the development of the skin carcinoma, but not the mucosal carcinoma in this patient.