verruciform xanthoma in indian literature has been described in the oral cavity but not from genital skin verruciform xanthoma ( vx ) is an uncommon clinicopathologic entity that primarily affects the oral and genital mucosa . clinically , it presents as a sessile , papillary , granular , or verrucous lesion that is often mistaken for a viral wart . collections of numerous foam cells in the elongated dermal papillae is a diagnostic feature of vx . shafer , in 1971 , described 15 cases of this condition in the oral cavity and coined the term verruciform xanthoma. following this first report , several cases were reported confirming vx as a distinctive clinicopathologic entity that presents clinically as a single verrucous or warty plaque and is characterized histologically by papillomatous epidermal hyperplasia with parakeratosis and the presence of neutrophils in the upper spinous layers . the histologic hallmark of this lesion , however , is the presence of numerous foam cells in the elongated dermal papillae . vx also occurs in nonoral sites and the first report of involvement of an extraoral site ( vulva ) was by santa cruz in 1979 . since then , several reports of vx in nonoral sites , especially on anogenital skin , have appeared in literature.[58 ] vx has a slight male preponderance , with a reported male : female ratio of 1.1 : 1 . in this paper review of biopsies received in consultation over the past 12 years ( 19992010 ) revealed five cases [ table 1 ] that fit in with the clinicopathological diagnosis of vx . all five cases were in males and presented with single , asymptomatic , verrucous / warty lesions that had gradually grown in size over several months . two cases occurred in the oral mucosa : one , about 2.5 cm in size , occurred on the left lower buccal mucosa at the level of the 1 and 2 left molar teeth [ figure 1 ] and the other on the anterior right margin of the tongue as a raised verrucous papule . both patients were smokers and case 1 also had history of chewing tobacco for 8 years before presentation . there was no bleeding from the lesions and no history of sensitivity to hot or spicy foods . the clinical diagnosis in the first case was leukoplakia , while in the second it was viral wart . verrucous plaque on the left buccal mucosa ( case 1 ) cases 3 and 4 developed single flat warty lesions on the penile skin , on the inner prepuce in case 3 and on the dorsum of the penis just proximal to the coronal sulcus in case 4 . case 1 was screened for serum lipids and all values were found to be within normal limits . biopsy findings in all five cases were similar and consisted of papillomatous and digitate epidermal hyperplasia , with some incurving of peripheral rete , giving the overall silhouette of a wart [ figure 2 ] . the upper spinous layers were infiltrated by several neutrophils that aggregated on the surface of the lesion [ figure 3 ] . the elongated dermal papillae were stuffed with foamy histiocytes with small central nuclei and abundant finely vacuolated cytoplasm [ figure 4 ] . parakeratosis was not evident in all cases but was very prominent in case 2 [ figure 5 ] , with surface and deep invaginations of columns of parakeratosis throughout the hyperplastic epidermis . exophytic papillomatous epidermal hyperplasia with silhouette of wart ( h and e ; 20 ) ( case 3 ) neutrophils in the upper spinous layers , with foamy histiocytes in the dermal papillae ( h and e ; 100 ) ( case 3 ) elongated dermal papillae stuffed with foam cells ( h and e ; 400 ) ( case 3 ) deep invaginated columns of parakeratosis ( h and e ; 40 ) ( case 2 ) features of human papilloma virus ( hpv ) infection , namely hypergranulosis and koilocytosis , were not seen in any of the biopsies . the lesions were excised , with no recurrence , in cases 1 and 2 ; the others cases were lost to follow - up . vx is an uncommon entity and we could find only two publications on the subject in indian literature ( a total of three cases , all in the oral cavity ) . the three cases of genital vx that we report are , to the best of our knowledge , the first report of nonoral vx from india . this may be due to lack of awareness of this condition or possibly reluctance to biopsy warty lesions of the genital mucosa and instead to treat them as viral warts / condylomata , which is the usual clinical diagnosis . on the basis histomorphologic appearance the epithelial changes of vx have been been divided into three groups  verrucous , papillary , and flat  and these epithelial changes reflect the clinical picture of the lesion . the diagnostic histological finding of all vx , however , whether intra- or extraoral in location , is the presence of foam cells that fill the dermal papillae and submucosal space . two aspects of this condition remain unresolved , namely the etiology and the pathogenesis of lipid - laden macrophages in the dermal papillae and submucosa . because the clinical appearance and histological silhouette closely resembles hpv - induced lesions like verrucae and condylomata and because the common sites of occurrence of vx is the oral and anogenital skin and mucosa ( sites common for hpv infection ) , hpv infection has always been a strong contender as the etiologic agent for this condition . most authors have not found any evidence for the presence of hpv in these lesions;[1214 ] however , contrary findings of multiple hpv dna in vx have also been reported . regarding the pathogenesis of lipid - laden macrophages in the dermal papillae , zegarelli proposed that initial damage to keratinocytes by an inciting agent is followed by degeneration of keratinocytes , which attracts dermal dendrocytes to engulf the debris and these develop into foam cells due to ingestion of lipids from the degenerating keratinocytes in the subepithelial dermis . the probable pathogenesis of vx has been postulated to be as follows :  following keratinocyte damage by an as yet unknown inciting agent , cytokines chemotactic for neutrophils are released , with rapid growth of the epidermis leading to the verruciform architecture and parakeratosis . the damaged and degenerating keratinocytes that move downwards into the papillary dermis and submucosal region are engulfed by dendritic cells , which develop into foam cells , losing in the process their normal histiocytic markers and stain negative for factor xiiia. xanthomatous changes in the papillary dermis , similar to that in vx , have been described in association with several diseases , including mycosis fungoides , epithelial nevi , and dystrophic epidermolysis bullosa . the changes may histologically resemble true vx and are considered to be a form of dystrophic xanthomatosis that possibly arises due to repeated epidermal or dermal damage , with accumulation of lipids within macrophages . simple excision of the lesion appears to be curative ; however , a 30-year - old woman with a recurrent vx of the vulva 8 years after initial treatment has been described in literature . to conclude , vx may be confused clinically with benign , premalignant , and malignant lesions like viral genital warts ( condylomata acuminata ) , leukoplakia , and verrucous carcinoma , and may even be associated with squamous cell carcinoma . genital warts induced by hpv are almost always multiple and therefore a single asymptomatic verrucous lesion on the oral or genital mucosa should always be subjected to biopsy to confirm the diagnosis and rule out malignancy . this series is , to the best of our knowledge , the first report of extraoral lesions of vx from india . we report these cases to increase awareness regarding this rather rare but distinctive condition that should be considered in the differential diagnoses of solitary verrucous lesion on mucosal sites , both oral and genital . nonoral ( genital skin ) verruciform xanthoma is described for the first time in indian cases .
we describe five cases of verruciform xanthoma ( vx ) . 
 the patients , all males , presented with single warty verrucous lesions of 0.52 cm size that had been diagnosed clinically as viral warts ( four cases ) and leukoplakia ( one case ) . 
 two patients had the lesion in the oral cavity , two on the genital mucosa , and one on the scrotal skin . 
 histopathology was diagnostic , with verrucous and papillomatous epidermal hyperplasia with the silhouette of a viral wart but with numerous foamy histiocytes packed in the elongated dermal papillae . 
 columns of deep parakeratosis and neutrophils in the upper spinous layers , with a dermal plasma cell infiltrate were the other histopathologic findings . 
 excision of the lesions was curative , without recurrences , in the two patients who had lesions in the oral cavity ; follow - up was not available in the cases with genital lesions . 
 vx is an uncommon but distinctive clinicopathologic entity affecting the oral and genital mucosa that may be mistaken for benign , premalignant , and malignant conditions . 
 vx can be diagnosed with certainty only on histopathologic examination .