疣状表皮发育不良伴Bowen病1例临床分析

Epidermodysplasia Verruciformis Accompanied by Bowen's Disease:A Case Report

目的:报道1例花斑癣型疣状表皮发育不良伴外阴部鲍温病,并对患者两处皮损的临床特点、病因及相关性进行分析。方法:将腰背部及外阴部皮损的石蜡包埋组织切片提取DNA后,使用凯普HPV37型基因分型试剂盒以及PCR扩增方法进行检测,同时对患者进行治疗。结果:两者试验结果未能体现两处皮损相关性,推测外阴皮损不是疣状表皮发育不良发展而来,为单独起病。患者T淋巴细胞明显降低。采用ALA-光动力治疗外阴皮损,三次光疗后皮损变化不明显,予以局部肿物完整切除术后,随访半年无复发。采用维A酸治疗疣状表皮发育不良皮损,治疗4个月后四肢疣状增生样皮损较前稍许变平,色素脱失斑片部分融合、边界较前模糊。结论:此病例伴发的鲍温病与疣状表皮发育不良无相关性;T淋巴细胞等因素可能在疣状表皮发育不良发病机制中占有作用。

Objective：A case of epidermodysplasia verruciformis presented with pityriasis-versicolor-like lesions and concomitant with Bowen＇s disease at vulva is reported.Pathogenesis and clinical characteristics of the patient were analyzed.Methods：A series of examinations were performed on the patient.DNA was extracted from paraffin-embedded tissue of truck and vulval lesions,then HPV detection was conducted with HPV37 gene analysis kits and PCR amplification was done with consensus primers.Medication was also prescribed for the patient.Results：Neither result of HPV detection or PCR amplification showed correlation between the lesions of truck and vulva.It seemed that the concomitant Bowen＇s disease in this case did not result from epidermodysplasia verruciformis.There was marked T lymphopenia in the patient.ALA-PDT treatment was applied to vulval lesions three times,but no obvious improvement was achieved.Complete excision was performed resulting in no recurrence at half-year follow-up.Retinoic acid was prescribed for treatment of epidermodysplasia verruciformis.Skin lesions of the limbs flattened and showed partial coalescence de-pigmented patches after 4 months of therapy.Conclusion：This study provides evidence that concomitant Bowen＇s disease is not related to epidermodysplasia verruciformis,and that T lymphocyte might play a part in the pathogenesis of epidermodysplasia verruciformis.