| Background Verruciform xanthoma(VX)is a rare benign proliferative disease that was first discovered in the mouth and named by Shafer in 1971.In addition to oral lesions,number of cases can involve the skin and vulva.VX can be a single disease,or coupled with other disease.The etiology and pathogenesis are unknown,and may be related to chronic inflammation,local irritation and repeated trauma,human papillomavirus infection,immune disorder,heredity,etc.However,it is generally considered to be independent of lipid metabolism.Recently,the number of VX cases reported in China has increased significantly,which is presumed to be related to the cognitive,clinical and pathological diagnosis of this disease.All ages can be involved,the incidence of male to female is 1.2: 1.80% cases were found in the oral mucosa,while 20% involved the vulva and skin.Single lesions are common,while multiple lesions are rare.It can be much rarer to find a case with oral,vulvar and skin involved.The common involvement of skin mucosa is in the vulva area(female labia,penis,foreskin,glans,sc rotum)and perianal skin etc.Clinically,the mucosa lesions are mostly skin color,reddish,yellowish red,pale yellow cauliflower,verrucous,papilloma-like lesions,and skin lesions are characterized by skin color,light yellow,yellowish brown,grayish yellow and thickened corners.The lesions vary in size and course(range from months to decades).VX is rare in clinical practice,and has various manifestations.It is difficult to diagnose based on clinical manifestations only.It is necessary to confirm the diagnosis by histopathological and immunohistochemical staining.At present,there is no standard treatment plan for this disease.Symptom targeted treatment,including surgical resection,laser treatment,local and systemic medication,etc.,are the most choice.However,for the generalized cases,because of the great psychological Stress,the treatment is still more difficult,deserving the high attention from clinicians.It is advisable to consider individualized conditions based on the patient’s specific circumstances and develop individualized treatment plans.Objective Collecting VX patient data published in domestic journals,and exploring the clinical features of rare VX involved in oral mucosa,including age,gender,clinical manifestations,affected sites,laboratory tests,histopathological changes,treatment methods and the outcome,in order to assist clinical data analysis reference for clinician diagnosis and treatment of VX.Methods 1.In term of " verruciform xanthoma " to search the literatures in "Chinese National Knowledge Infrastructure(CNKI)","Wanfang database";2.Screen the retrieved documents based on inclusion/exclusion criteria: Inclusion criteria:(1)Previous literature related to VX included in China Knowledge Network and Wanfang database,in addition to oral involvement,lesions also involve skin and other mucosal sites,or lesions only involve skin or mucosal sites outside the mouth;(2)confirmed by histopathological examination;(3)complete medical records,including age,sex,clinical manifestations,the location of the disease,laboratory tests,treatment methods and treatment outcomes;Exclusion criteria:(1)VX only affects the previous literature of the oral cavity;(2)previous literature on missing case data;3.18 articles and a total of 21 cases were included,including 1 case in our hospital.Deduce and summarize the clinical data.Results 1.General information 47 articles related to VX were obtained by searching the Chinese Knowledge Network and Wanfang database with the scorpion xanthomas as the key words.Of them,9 articles of simple oral mucosa,12 of reviews,and 8 with incomplete medical records were excluded according to the exclusion criteria.The other 18 articles,reporting 21 cases,were included.13 males,8 females,including 1 case of our hospital.The onset age ranges from 2 years to 81 years,and the median onset age is 34 years old.The course ranges from 2 weeks to 32 years.71.43%(15/21)patients had single lesions.28.57%(6/21)patients had multiple lesions,of which 50.00%(3/6)were single site involvement,50.00%(3/6)were ≥2 affected sites,mainly in the vulva,mouth and skin.75.00%(18/21)showed single or multiple lesions of different sizes in the vulva.9.52%(2/21)involved both vulva and skin.4.76 %(1/21)with oral-vulvar-skin involved was a rare disseminated VX.All 21 cases involved the vulva,while 28.57%(6/21)involved the scrotum,19.05%(4/21)involved the foreskin,14.29%(3/21)involved the glans,23.81%(5/21)involved the female genitals,9.52%(2/21)involved both vulva and skin,and 4.76%(1/21)with vulva,mouth and skin all involved.4.76 %(1/21)had a history of traumatic lesions in the lesion area,4.76 %(1/21)with HPV infection,4.76 %(1/21)with a history of visiting prostitutes,and 14.29%(3/21)with phimosis.4.76%(1/21)with family member had the same history.2.Clinical features(1)Mucosal manifestation 4.76%(1/21)VX involve the oral mucosa,which is characterized by multiple pink patches or verrucous of varying sizes of gums,tongues,and hard palate.47.62%(10/21)of the VX,involving the glans and vulva mucosa,showed white,light yellow,brownish yellow,reddish cauliflower-like or sputum plaques or nodules.(2)cutaneous manifestations 57.14%(12/21)VX,involving the vulva skin,manifested as single or multiple cauliflower-like,papillary or scorpion-like pedicles or sessile The red,skin color or yellow-red nodules of the pedicle in the foreskin,scrotum,perianal,penis,and female genital skin,some of which are susceptible to bleeding,one of which with a large lesion was diagnosed as a huge scrotal xanthoma.14.29%(3/21)of the skin VX,manifestated as a single or multiple skin color,yellow-brown,reddish-brown surface with a rough or keratinized papule or nodule with varying size on the face,trunk or limbs.Lesions of 2 cases with multiple lesions presented with linear appearance.(3)Other manifestation 4.76%(1/21)felt a slight pain.9.52%(2/21)felt itch.4.76%(1/21)was susceptible to bleed.9.52%(2/21)with partial urethra covered may present as urinary rest and urinary tract obstruction,which was not mentioned in previous literatures.9.52%(2/21)had wet surface coupled with odor secretions.3.Histopathological finding Microscopic findings include the papilloma-like hyperplasia of the epidermis,hyperkeratosis with parakeratosis,epithelial foot extension,foam cell aggregation in the dermal papilla,superficial vascular hyperplasia,dilatation,congestion.A small amount of chronic inflammatory cells,such as neutrophils,lymphocytes and plasma cells,infiltrate the dermis.Immunohistochemical staining of foam cells were CD68(+)and S100(-).4.Treatment and prognosis Of 21 patients,71.43%(15/21),underwent complete surgical resection,were cured;4.76%(1/21)were treated with liquid nitrogen freezing combined with surgery;4.76%(1/21)healed after liquid nitrogen cryotherapy.Multiple liquid nitrogen treatment was ineffective in 4.76%(1/21)cases,while topical imiquimod realized the significant improvement.After 9 months of withdrawal,no new lesions were seen,and histological examination proved that the foam cells were completely disappeared.9.52%(2/21)were given multiple CO2 laser treatments,a case was cured while the other was ineffective.4.76%(1/21)CO2 were given laser combined with surgery,oral Avi A Capsules,topical adapalene gel and 0.03% tacrolimus ointment,which has been proved effective.All in all,85.71%(18/21)were cured.There was no recurrence in the follow-up period from 3 months to 3 years.9.52%(2/21)was effective,and 4.76%(1/21)was ineffective.Conclusions 1.VX can always be found involving the oral,vulva or skin,separately,while more than 2 parts involved in a man is rare.2.Surgical resection was the main choice,assisted with local laser treatment.The prognosis is good,and there is little recurrence;Avi A orally,adapalene,imiquimod,tacrolimus for external treatment is also effective. |
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