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Gardner-Diamond syndrome is a rare skin disease characterized by recrudescent, spontaneous and tender ecchymoses, and mostly affects psychologically disturbed adult women. The positive autoerythrocyte sensitization test has noticeable diagnostic significance. Currently, there is no specific therapy for Gardner-Diamond syndrome. This review summarizes advances in the pathogenesis, clinical features, diagnosis and treatment of this disease.
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Unlike the traditional surgical approach, which requires priority treatment of the perihepatic ligament and rotation of the liver, the anterior approach to the right hemicolectomy does not deal with the perihepatic ligament prior to parenchymal dissection and rotate and move the liver. The advantage is that it is more in line with the tumor-free principle and reduces unnecessary free. The disadvantage is that it is relatively difficult to control bleeding during the cleavage of the liver and there is some risk in the handling of the lower part of the vena cava sulcus. However, with the popularization and application of laparoscopy, it is more suitable for performing anterior approach right hemicolectomy than open surgery due to its unique caudodorsal view advantage, finer local anatomical structure display and subtle manipulation. This article discusses the procedures and technical points of laparoscopic right hemihepatectomy through the anterior approach.
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Objective: Network pharmacology combines drug and disease targets with biological information networks based on the integrity and systematicness of the interactions between drugs and disease targets. This study aims to explore the molecular basis of Hanshi Zufei formula for treatment of COVID-19 based on network pharmacology and molecular docking techniques. Methods: Using TCMSP, the chemical constituents and molecular targets of Atractylodis Rhizoma, Citri Reticulatae Pericarpium, Magnoliae Officinalis Cortex, Pogostemonis Herba, Tsaoko Fructus, Ephedrae Herba, Notopterygii Rhizoma et Radix, Zingiberis Rhizoma Recens, and Arecae Semen were investigated. The predicted targets of novel coronavirus were screened using the NCBI and GeneCards databases. To further screen the drug-disease core targets network, the corresponding target proteins were queried using multiple databases (Biogrid, DIP, and HPRD), a protein interaction network graph was constructed, and the network topology was analyzed. The molecular docking studies were also performed between the network's top 15 compounds and the coronavirus (SARS-CoV-2) 3CL hydrolytic enzyme and angiotensin conversion enzyme II (ACE2). Results: The herb-active ingredient-target network contained nine drugs, 86 compounds, and 49 drug-disease targets. Gene ontology (GO) enrichment analysis resulted in 1566 GO items (P < 0.05), among which 1438 were biological process items, 35 were cell composition items, and 93 were molecular function items. Fourteen signal pathways were obtained by enrichment screening of the KEGG pathway database (P < 0.05). The molecular docking results showed that the affinity of the core active compounds with the SARS-CoV-2 3CL hydrolase was better than for the other compounds. Conclusion: Several core compounds can regulate multiple signaling pathways by binding with 3CL hydrolase and ACE2, which might contribute to the treatment of COVID-19.
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Objective To analyze the disease constitution,accuracy of clinical and pathological diagnoses of skin biopsy samples in Peking Union Medical College Hospital.Methods A total of 29987 patients subjected to skin biopsy were collected from Department of Dermatology,Peking Union Medical College Hospital from June 2010 to November 2018,and clinical and histopathological diagnoses of these skin biopsy samples were analyzed retrospectively.Results According to the results of histopathological diagnosis,confirmed diagnoses of these patients could be classified into 33 categories and 242 kinds.Common disease categories included epidermal tumors (2931 cases,9.77%),connective tissue diseases (2809 cases,9.37%),melanocytic tumors (2078 cases,6.93%),erythematous scaly pustular dermatoses (1376 cases,4.59%),lichenoid dermatoses (1291cases,4.31%),allergic or eczematous skin diseases (1282 cases,4.28%)and infectious skin diseases (1156 cases,3.86%).Common skin diseases included scleroderma (1887 cases,6.29%),pigmented nevus (1755 cases,5.85%),seborrheic keratosis (1136 cases,3.79%),eczema (1089 cases,3.63%),psoriasis (881 cases,2.94%),lichen planus (867 cases,2.89%),lupus erythematosus (638 cases,2.13%),pemphigus (549 cases,1.83%),and basal cell carcinoma (501 cases,1.67%).Poor consistency was observed between clinical diagnosis and histopathological diagnosis of lichen planus,bullous pemphigoid,granuloma annulare and hypereosinophilic dermatitis.Conclusions Common disease categories of the skin biopsy samples in Peking Union Medical College Hospital were epidermal tumors,connective tissue diseases,melanocytic tumors,erythematous scaly pustular dermatoses,lichenoid dermatoses,and allergic or eczematous skin diseases.Poor consistency was observed between clinical and pathological diagnosis in some skin diseases,and understanding of these diseases should be improved.
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Inflammasomes are a kind of intracellular multiprotein complex that comprises a part of the innate immune response. Inflammasome function abnormalities can lead to immune imbalance and have been linked to an increasing number of diseases. Researches have shown that inflammasomes are associated with some skin tumors including basal cell carcinoma, squamous cell carcinoma, Kaposi′s sarcoma and malignant melanoma, implicating that persistent inflammatory response and immune imbalance caused by abnormal activation of inflammasomes play a crucial role in tumor occurrence, development, invasion and metastasis. This review summarizes research advances in the role of inflammasomes in the pathogenesis of skin tumors.
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OBJECTIVE:To establish the fingerprint of ethanol extract and acetone extract from Anemarrhena asphodeloides and its different processed products ,and to investigate the spectrum-effect relationship between the fingerprint and the antioxidant activity. METHODS :HPLC method and HPLC-ELSD method were adopted. The determination was performed on Thermo BDS Hypersil C 18 column with mobile phase consisted of acetonitrile- 0.2% acetic acid at the flow rate of 1.0 mL/min. The column temperature was 30 ℃,and the detection wavelength was set at 258 nm. The sample size was 10 μL. The determination was performed on XDB-C 18 columnwith mobile phase consisted of acetonitrile-0.1% acetic acid (gradient elution )at the flow rate of 0.9 mL/min. The column temperature was 30 ℃ . The temperature of atomizer was 40 ℃ and the flow rare of N 2 was 1.6 mL/min. The sample size was 10 μL. Using mangiferin and timosaponin B Ⅱ as reference ,Fingerprint Similarity Eva- com luation System of TCM Chromatogram (2004A edition )was adopted to draw the fingerprint of ethanol extract and acetoneextract from 20 batches of A. asphodeloides and its different processed products to confirm common peaks. Using scave nging rate of 1,1-diphenyl-2-trinitrophenylhydrazine(DPPH)radical as index,antioxidant activities of ethanol extract and acetone extract from 20 batches of A. asphodeloides and its processed products were investigated. Using scavenging rate of DPPH radical as dependent variable ,common peak area as independent variable ,PLSR was used to analyze the spectrum-effect relationship of ethanol extract and acetone extract from A. asphodeloides with antioxidantion activity. RESULTS :Eight peaks (M1-M8)were identified in the fingerprints of ethanol extracts from 20 batches of processed A. asphodeloides . Mangiferin (chromatogram peak M 7)was identified with similarity of 0.389-1.000;seven comon peaks (S1-S7)and timosaponin B Ⅱ(peak S 5)were identified in the fingerprint of acetone extract ,and the similarity was 0.044-0.999. DPPH radical scavenging rate of ethanol extract from 20 batches of A. asphodeloides and its processed products was 21.23%- 81.39%,and A. asphodeloides was significantly lower than salt-processed A. asphodeloides with salt wine-processed A. asphodeloides (P<0.001);and that of acetone extract was 49.73%-83.78%,and A. asphodeloides was significantly higher than stir-baked A. asphodeloides with salt ,wine or fire (P<0.001). The standardized regression coefficients of peaks M 2-M7 in the spectrum of ethanol extract from A. asphodeloides were all greater than 0,which was positively correlated with antioxidant activity. Only the variable importance projection (VIP)value of peak M 7 was greater than 1,which had an important contribution. The standardized regression coefficients of peaks S 4-S7 in the acetone extract spectrum of A. asphodeloides were greater than 0,and were positively correlated with antioxidant activity. The order of VIP values was peak S 5>S6>S4,and the VIP values were all greater than 1. CONCLUSIONS:The fingerprint of the different processed products A. asphodeloides and its antioxidant activity spectral effect relationship were successfully established ;mangiferin(peak M 7)may be the main antioxidant substance of ethanol extract from A. asphodeloides . Timosaponin B Ⅱ(peak S 5),peak S 6 and peak S 4 may be the main antioxidant substance in acetone extract from A. asphodeloides .
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As an effective treatment method of tumors and other diseases,radiotherapy has been widely applied to clinical practices.After radiotherapy,about 95% of patients suffer from skin injuries to different extents,including acute and/or chronic radiodermatitis,or radiotherapy-related skin diseases,including cutaneous fibrosis,morphea,sclerosing panniculitis,bullous skin diseases,eosinophilic polymorphic and pruritic eruption,lichen sclerosus et atrophicus,angiosarcoma,atypical vascular lesions and radiation recall dermatitis.These skin injuries greatly affect the quality of life and treatment compliance of patients.This review briefly summarizes common and rare skin diseases induced by radiotherapy,so as to improve the understanding of radiotherapy-related skin diseases in clinicians.
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Autoinflammatory diseases are a group of inherited disorders characterized by dysregulation of the immune system,periodic fever and reccurrence of inflammation.The common rashes of autoinflammatory diseases include acne,pyoderma gangrenosum,erysipelas,urticaria,ichthyosis,pustulosis,while vasculitis is rare.This review summarizes autoinflammatory diseases accompanied by vasculitis.Familial Mediterranean fever mainly manifests as small-and medium-vessel vasculitis such as Henoch-Sch(o)nlein purpura and polyarteritis nodosa,while large-vessel vasculitis is common in Blau syndrome.Small-and medium-artery vasculitis can occur in patients with deficiency of adenosine deaminase 2 as well,so it should be differentiated from polyarteritis nodosa in clinical practice.Stimulator of interferon gene-associated vasculopathy with onset in infancy mainly presents as cutaneous small-vessel vasculitis and vasculopathy.
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OBJECTIVE: To establish a method for simultaneous determination of spinosin and jujuboside A in the seads of Ziziphus jujuba, and to investigate its quality grading standard. METHODS: HPLC-ELSD method was adopted. The separation was carried out on Inertsil ODS-SP column with mobile phase consisted of acetonitrile-water (gradient elution) at the flow rate of 1.0 mL/min. The column temperature was 30 ℃, the temperature of drift tube was 90 ℃, the flow of carrier gas was 2.9 L/min and injection volume was 20 μL. The thickness, width, length and 100-grain quality of the medicinal materials were used as indicators to investigate the appearance traits. SPSS 22.0 software was used to analyze the correlation of the contents of spinosin and jujuboside A, its appearance traits with the quality constant of TCM, and establish a quality classification standard for the seads of Z. jujuba. RESULTS: The linear range of spinosin and jujuboside A were 1.03-6.18 μg/mL (r=0.999 7), 1.05-6.30 μg/mL (r=0.999 8); the limits of quantitation were 0.171, 0.174 μg/mL, respectively; the limits of detection were 0.052, 0.053 μg/mL, respectively. RSDs of precision, stability and reproducibility tests were all lower 2%. The recoveries were 99.01%-102.97% (RSD=1.39%, n=6), 97.94%-101.03% (RSD=1.13%, n=6), respectively. Correlation analysis results showed that the length, width, 100-grain quality spinosin content and jujuboside A content of the medicinal materials were positively correlated with the quality constant of TCM. The results of quality classification for 30 batches of medicinal materials showed that S1-S4 and S7-S12 were first-class products; S5, S6, S13-S17 and S20-S30 were second-class products; S18 and S19 were third-class products. CONCLUSIONS: Established content determination method is simple, precision, accurate and stable, and can be used for simultaneous determination of spinosin and jujuboside A in the seads of Z. jujuba. Established quality grading standard of the seads of Z. jujuba can be used to evaluate the quality.
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On November 30th,2017,a 62-year-old female patient was admitted to the hospital because of multiple depigmented patches all over the body for 16 years,as well as erythema and blisters on the scalp,trunk and upper extremities for more than 8 years,which had been aggravated for 1 year.The patient underwent surgical resection of thymoma in 1996,and developed myasthenia gravis in 2004.Skin examination showed scattered erythemas of various sizes on the scalp,face,chest and upper extremities,with crusts on the surface of some erythemas and positive Nikolsky's sign.Hypopigmented macules mingled with depigmentated macules of various sizes were scattered all over the body.A large area of depigmentation was observed on both hands,and the nail plates were atrophic.Hairs,eyebrows,eyelashes,axillary and pubic hairs were abscent.Laboratory examination showed the presence of homogeneous IgG antinuclear antibody with a titer of 1:640,anti-double stranded DNA antibody (276 IU/ml),lupus anticoagulant (1.62),anti-Dsg1 antibody (> 150 U/ml) and anti-Dsg3 antibody (91 U/ml).Histopathological examination of dorsal blister tissues and hematoxylin-eosin staining showed fissured blisters and acantholytic cells in the epidermis,monolayer basal cells on the basilar membrane,and perivascular infiltration of a few lymphocytes in the superficial dermis.The patient successively developed thymoma,vitiligo,myasthenia gravis,systemic lupus erythematosus,pemphigus vulgaris and alopecia universalis,so a diagnosis of autoimmune polyglandular syndrome type Ⅳ was made.Since autoimmune-mediated endocrinopathies are prone to occur successively,changes of related hormones and antibodies in the patient should be monitored for early diagnosis and treatment of newly emerging autoimmune diseases.
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Objective To investigate the dermoscopic features of vulvar lichen sclerosus (VLS).Methods Seventeen patients with VLS,who visited the Department of Dermatology of Peking Union Medical College Hospital between August 2015 and June 2017 and underwent dermoscopy and skin histopathological examination,were enrolled into this study.The dermoscopic images of 75 skin lesions were collected and analyzed retrospectively.Results The common dermoscopic features in these VLS lesions included yellowish-white structureless areas (89.3%,67/75),reddish-violet globules or patches (77.3%,58/75),brownish or bluish-gray pigment structures (49%,37/75),shiny white streaks (40%,30/75),dotted vessels with irregular arrangement (28%,21/75),linear vessels (58.7%,44/75),hairpin-like vessels (6.7%,5/75),comma-like vessels (13%,10/75)and branching vessels (20%,15/75).Under dermoscopy,rosette sign could be observed in 3 (4%) lesions,comedo-like openings in 3 (4%) lesions,and keratotic plugs in 1 (1%) lesions.Conclusion Under dermoscopy,VLS is characterized by yellowishwhite structureless areas,reddish-violet globules or patches,pigment structures and vessels with various vessels.
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Two cases of frontal fibrosing alopecia (FFA) were reported.Case 1,a 44-year-old woman,presented with progressive recession of the frontotemporal hairline for 4 years and multiple skincolored facial papules for 2 years.Skin examination showed that the frontotemporal hairline was receded,the local skin was smooth and thin,and a few remaining fine hairs could be seen.Additionally,eyebrows,axillary and pubic hairs were partly shed,and plenty of millet-sized skin-colored papules were diffusedly distributed on the frontotemporal region and bilateral mandibular angles.Dermatoscopy showed reduced hair follicular openings,different hair shaft diameters,cicatricial white patches and perifollicular erythema.Case 2,a 55-year-old woman,presented with progressive frontotemporal hair loss for 2 years.Skin examination revealed the recession of bilateral frontotemporal hairline and partial loss of eyebrows,axillary and pubic hairs.Histopathological examinations of the 2 patients both revealed perifollicular infiltration of lymphocytes,liquefaction degeneration of basal cells and perifollicular lamellar fibrosis.Clinical manifestations and histopathological features of the 2 patients both confirmed the diagnosis of FFA.
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Objective To detect the changes of cell invasion and proliferation in hepatocellular carcino -ma cell line HepG2 through the upregulation of miR -222.Methods The expression of miR-222 was up-reg-ulated by liposomal transfection .The expression of PTEN which is the targeting protein of miR -222 was detected by using Western Blot assay .The changes of proliferative capacity and invasive ability in HepG 2 cells after the upregulation of miR-222 were detected through MTT assay and Transwell assay .Results In Western Blot ex-periments,the expression of PTEN in transfection groups were lower than that in negative control groups and blank control groups.In MTT assay,the OD values in transfected group were higher than that in negative control group and blank control group(P<0.05).In transwell experiments,the number of penetrating cells in transfected group were higher than that in negative control group and blank control group (P<0.05).Conclusion Our results sug-gest that PTEN is significantly down regulated by the upregulation of miR -222.The proliferative capacity and inva-sive ability are raised in hepatocellular carcinoma cell line HepG 2 through the upregulation of miR-222.
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<p><b>BACKGROUND</b>The World Health Organization and European Organization for Research and Treatment of Cancer (WHO-EORTC) classification in 2005 promoted the comparisons of primary cutaneous lymphoma (PCL) subtypes between different countries. The relative frequency of PCL varied according to geography. The study aimed to analyze the relative frequency and survival of PCLs in China and to compare the data with the published results from other countries.</p><p><b>METHODS</b>We analyzed 98 patients with PCLs over a 6-year period and reclassified them according to the most recent WHO-EORTC classification (2005). Disease-specific survival rate and curves according to specific subtypes such as mycosis fungoides, lymphomatoid papulosis, and primary cutaneous peripheral T-cell lymphoma, unspecified was also calculated.</p><p><b>RESULTS</b>The relative rate of PCL in China was distinct from those in Western countries. Our study showed a higher frequency of cutaneous T- and NK-cell lymphomas (CTCLs) (94%), and a lower frequency of cutaneous B-cell lymphomas (CBCLs) (6%). The 5-year survival rate of the total PCLs was 82%. There was no significant difference in the 5-year survival rate (P > 0.05 by Log-rank test) between CTCL (80%) and CBCL (100%).</p><p><b>CONCLUSIONS</b>The higher percentage of CTCL in China may provide a clue to further study the etiological factors of PCLs. Racial variations in factors such as HLA determinants may play a role in the development of CTCL.</p>
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Adolescent , Adult , Aged , Aged, 80 and over , Child , Female , Humans , Male , Middle Aged , China , Epidemiology , Lymphoma , Epidemiology , Mortality , Retrospective StudiesABSTRACT
Three cases of medallion-like dermal dendrocyte hamartoma are reported.The patients included two men and one woman,and were aged from 2 to 38 years.All the patients presented with medallion-shaped,welldefined,slightly atrophic and asymptomatic congenital patches with prominently dilated superficial veins that had grown proportionately with the body length.Histological examination revealed similar changes in all of these patients,including epidermal atrophy and dermal proliferation of spindle cells,which stained positive for CD34,factor ⅩⅢa and vimentin,but negative for S100,CD68,CDla,CD31 and smooth muscle actin (SMA).None of the patients received special therapy,and the lesions enlarged slightly with body growth within 3-4 years of follow-up after the diagnosis.
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A case of the H syndrome was reported.An 18-year-old man presented with an 8-year history of progressive cutaneous sclerosis,skin hyperpigmentation,and hypertrichosis on the trunk and extremities.In addition,he suffered from short stature,hearing loss,cardiac anomaly,hepatosplenomegaly,and scrotal masses.Laboratory examination revealed hypogonadism.Cutaneous histopathologic examination showed hyperpigmentation of the basal layer with acanthosis,widespread fibrosis (moderate in dermis and severe in subcutis) and an infiltrate composed of histiocytes and plasma cells.A perivascular lymphocyte infiltrate was also observed throughout the dermis and subcutaneous fat tissue.These findings were consistent with the H syndrome.Differential diagnosis must be made with Winchester syndrome and polyneuropathy, organomegaly,endocrinopathy,M-protein and skin change (POEMS) syndrome.
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Objective To evaluate the efficacy and safety of 0.03% tacrolimus ointment for the treatment of atopic dermatitis (AD) in Chinese children. Methods A total of 139 children, 2 to17 years of age, with moderate to severe AD from 5 study centres were enrolled in this multicentre, randomized, double-blind, vehicle-controlled, parallel group study. Treatment with 0.03% tacrolimus ointment or vehicle was applied twice daily to the affected areas for 3 weeks. Visits were scheduled on day 1 (base line, before treatment) and 1, 2, 3 weeks after the treatment. The main therapeutic parameter was the efficacy rate at the end of the treatment. Results The efficacy rates were 84.6% and 29.0% for tacrolimus group and vehicle group, respectively (P
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Objective To analyse gene mutation in members of a Chinese family with Hailey-Hailey disease(HHD)and study the relationship between the genotype and clinical features of the disease.Meth-ods Genomic DNA of leucocytes were obtained from members of the Chinese family with HHD including4patients and6normal persons.Ten exons of ATP2C1gene were amplified by polymerase chain reaction(PCR)and the products were analysed by single-strand conformation polymorphism(SSCP)and direct DNA sequencing.Results A novel mutation was identified in this family.The sequence of"TGTAGCCAT"(2068→2076)was substituded by"AGATGGAACA",which caused a frame shift of open reading frame and premature termination codon(PTC)in gene ATP2C1.There was no relationship between the genotypes and the phenotypes.Conclusion Gene mutation of ATP2C1gene at exon21is the cause for HHD in this fami-ly.
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Objective To induce the mutation of HPV-16 E7 in two zinc-binding motifs near the C terminus by polymerase chain reaction (PCR) and evaluate the effect of this mutation on the antigen-specific immunity of HPV-16 E7. Methods HPV-16 E7 fragment was amplified by PCR and cloned into pGEM-3zf vector. Two site mutations at 58 and 91 animo acid sites in the open reading frame of HPV-16 E7 were induced by PCR, and then the molecular clones of HPV-16 E7 wild type (pcDNA3.1/E7) and mutant (pcDNA3.1/ME7) were successfully reconstructed. Western blot and immunofluorescence were used to detect the expression of E7 protein. Results Intracellular fluorescence signals were observed in the cells transfected with pcDNA3.1/E7 and pcDNA3.1/ME7 24 hours after transfection, but the signals in the cells transfected with pcDNA3.1/ME7 disappeared 48 hours after tansfection. Twenty-four and 48 hours after transfection with pcDNA3.1/ME7, E7 protein was not detected by Western blot. Conclusions The stability of HPV-16 E7 protein is reduced by mutations (C58G, C91G) near two zinc-binding motifs. It is suggested that the zinc-binding motifs near the C terminus of HPV-16 E7 may be important for maintaining the stability of E7 protein.
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Objective To study the possible relationship between human papillomaviruse (HPV) infection and external genital proliferative diseases. Methods HPV DNA was detected using polymerase chain reaction (PCR) with a consensus primer and typed by using restriction fragment length polymorphism (RFLP) method in 151 paraffin-embedded biopsy specimens from seven kinds of external genital epidermal proliferative lesions. Results In 30 cases of condyloma acuminatum, HPV DNA was positive for all cases, among which HPV6 and HPV11 accounted for 60% and 26.7%, respectively. In 40 cases of bowenoid papulosis, 5 cases of genital Bowen′s disease and 6 cases of erythroplasia of Queyrat, the positive rates of HPV DNA were 55%, 100% and 33.3%, respectively, and the predominant type was HPV16. In 18 cases of external genital invasive squamous cell carcinoma HPV DNA positive rate was 27.8%, and HPV16 was detected in all of the 5 HPV-positive cases. HPV DNA was negative in 32 cases of leukoplakia and 20 cases of extramammary Paget′s disease. Conclusions Condyloma acuminatum is mainly caused by HPV6 infection, followed by HPV11; HPV16 infection is closely associated with the pathogenesis of bowenoid papulosis and external genital squmous cell carcinoma including Bowen′s disease and erythroplasia of Queyrat. In external genital invasive squamous cell carcinoma, HPV16 infection may be one of the multiple carcinogenic factors. No clear relationship is found between HPV infection and leukoplakia or extramammary Paget′s disease.