| BackgroundEpidermodysplasia verruciformis(EV)was first proposed in 1922,is considered as a kind of with EVER1 or EVER2 mutations in autosomal recessive hereditary disease,in recent years,with the deepening of the epidemiological,virological and clinical research,found that some patients have other except EVER1,EVER2 gene mutation or secondary to other immune system damage,now verrucous epidermal dysplasia is divided into the classic genetic type,not the classic genetic type and type 3 classes.Some patients with verrucous epidermal dysplasia are associated with skin tumors.ObjectiveThe purpose of this paper is to analyze the characteristics,clinical manifestations,histopathology,secondary diseases,treatment methods and prognosis of this disease,so as to improve the overall understanding of clinicians on this disease,provide experience for clinical treatment,so as to early detection,early intervention,and improve the prognosis of patients.MethodsCollection of qilu hospital of shandong university in July 2017 to December 2019 hospitalized cases of verrucous epidermal dysplasia diagnosed by 2 cases,retrieved from CNKI,PUBMED,ten thousand related documents in the database,and the key for domestic and foreign literature in recent 20 years,154 cases of data more complete verrucous epidermal dysplasia in patients with clinical data were retrospectively analyzed,characteristics of verrucous epidermal dysplasia pathogenesis,clinical manifestation,histopathological manifestations,secondary disease,concurrent disease,auxiliary examination,treatment and prognosis were summarized and analyzed retrospectively.The patients with combined skin tumors were divided into separate groups to summarize the site of disease,clinical manifestations,disease course and prognosis of patients with combined skin tumors.ResultsPart 1:The clinical data of 2 patients with verrucous epidermal dysplasia admitted to our hospital are as follows:1.General information:all the 2 cases were male,with an onset age of 30-55 years and an average onset age of 42.5 years.The course of disease was 9-20 years,with an average course of 14.5 years.2.Clinical manifestations and distribution of lesions:1 case presented with verrucous flat and hypertrophic plaque lesions,and 1 case presented with verrucous flat and macular floral lesions.All the 2 cases had systemic generalized hair,no associated symptoms and no nail damage.3.Family history:1 case with family history:the parents were closely related,but the children had no similar behavior.1 case had no family history.4.Laboratory examination:2 cases showed abnormal t-lymphocyte subsets,and other abnormal results included blood glucose,blood lipid and IgE5.Skin tumor complicated with basic diseases:1 patient with history of type 2 diabetes and hypertension,combined with squamous cell carcinoma and Bowen’s disease;There was 1 case of squamous cell carcinoma with no underlying disease.6.Treatment:1 patient underwent skin grinding and cryotherapy,oral tretinoic acid treatment,1 patient underwent cryotherapy and oral tretinoic acid treatment.7.Prognosis:1 case improved with reduced skin lesions.One case had no obvious effect,and the axillary lymph nodes were enlargedPart 2:Literature review and data analysis1.The general information Among the 154 patients in this study,91 were males and 63 were females,with a male-female ratio of 1.44:1 and an average age of 15.99±13.7 years.The mean age of EV patients was 11.6±8.7 years,and that of AEV patients was 28.7±19.0 years.There were 46 cases of skin tumor,the incidence of which was about 38.0%in patients with genotype EV.The average course of disease from EV clinical symptoms to the first skin tumor was 21.5±11.3 years.2.Clinical manifestations and distribution of skin lesionsAmong the 154 patients in this study,flat verrucous type was the most common,and 141 patients had flat verrucous skin lesions,with a percentage of 91.6%.Among them,9 patients complained of itchy symptoms and 7 patients developed part a symptoms.In this study,9 cases had misdiagnosis experience.Among the 46 patients with skin tumors,the most common tumor sites were on the head and face,Ulcer is the most common clinical manifestation.3.Histopathological examinationHistopathological examination of verrucous epidermal dysplasia showed many characteristics of verrucous lesions.The lesions were characterized by hyperkeratosis,keratosis,acanthosis with eosinophilia and cytoplasmic eosinophilia.4.Family history and genetic testingIn this study,among the 121 patients with hereditary EV,38(31.4%)had family history,among whom AVE patients had no positive family history.5.Auxiliary examinationThe main examination items were blood routine,urine routine,stool routine,liver and kidney function,T lymphocyte subgroup,and HIV and hepatitis series.Some patients with pityriasis pityriformis were tested for fungus and all were negative.6.Secondary and concurrent diseasesThe most secondary diseases were squamous cell carcinoma(35 cases),and the other major secondary diseases were basal cell carcinoma(BCC).Solar keratosis,Bowen’s disease.In patients with hereditary EV,hepatitis is the most common complication.In AEV patients,the comorbiditie disease is an infection,such as HIV,or the use of immunosuppressive agents.7.HPV typesIn this group,55 patients were tested for HPV,and the main types were:17 patients were positive for hpv-5,9 were positive for hpv-14,7 were positive for hpv-8,4 were positive for hpv-23 and 25,3 were positive for hpv-16 and 20,and 2 were positive for hpv-2 and 17.8.Treatment and prognosisAmong the patients in this study,93 cases were recorded with treatment regimens,of which 50 underwent surgical resection.Twenty-seven cases were treated by oral administration of avir A.Routine physical therapy such as freezing and C02 laser.Follow-up after treatment was recorded in 48 cases,of which 37 cases showed varying degrees of improvement.1 case of recurrence and 10 cases of death.Conclusions1.Epidermodysplasia verruciformis can occur at any age,among which the genetic verrucous epidermal dysplasia onset age is earlier,while the acquired verrucous epidermal dysplasia onset age is relatively later.2.The clinical manifestations of epidermodysplasia verruciformis are various,among which flat warts are the most common,and often exist in multiple types at the same time.3.Skin tumors secondary to epidermodysplasia verruciformis occurred at the site of exposure,among which squamous cell carcinoma was the most common,followed by basal cell carcinoma and Bowen’s disease.4.EV patients should choose appropriate treatment methods or a combination of multiple methods according to their conditions.The primary lesions are mainly oral and topical retinoids,and the secondary lesions are mainly surgical excision.5.Early detection,regular review and daily sun protection help to improve the prognosis of patients. |
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