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An Official Publication of the Indian Association of Oral and Maxillofacial Pathologists


 
CASE REPORT Table of Contents   
Year : 2008  |  Volume : 12  |  Issue : 1  |  Page : 41-44
 

Verruciform xanthoma: Report of two cases and review on pathogenesis


Department of Oral and Maxillofacial Pathology and Microbiology, Institute of Dental Sciences, KLE University, Belgaum - 590 010, India

Correspondence Address:
Kunal Sah
Department of Oral and Maxillofacial Pathology and Microbiology, Institute of Dental Sciences, KLE University, Belgaum - 590 010, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-029X.42198

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   Abstract 

Verruciform xanthoma [VX] is an uncommon benign mucocutaneous lesion of unknown etiology. This rare, harmless lesion usually presents as sessile or pedunculated, pale yellowish-to-red, papillary, granular or verrucous mucosal growth. Histologically VX is characterized by the presence of parakeratinzed epithelium showing papillary or verrucous growth with thin rete ridges and connective tissue papillae extending up to the surface. The papillae characteristically consist of foam cells, also called xanthoma cells. We report two cases of verruciform xanthoma and discuss their clinical and histopathological findings.


Keywords: Pathogenesis, verruciform xanthoma


How to cite this article:
Sah K, Kale AD, Hallikerimath S. Verruciform xanthoma: Report of two cases and review on pathogenesis. J Oral Maxillofac Pathol 2008;12:41-4

How to cite this URL:
Sah K, Kale AD, Hallikerimath S. Verruciform xanthoma: Report of two cases and review on pathogenesis. J Oral Maxillofac Pathol [serial online] 2008 [cited 2020 Nov 27];12:41-4. Available from: https://www.jomfp.in/text.asp?2008/12/1/41/42198



   Introduction Top


Verruciform xanthoma [VX] is an uncommon benign lesion primarily of the oral mucosa, first described by Shafer in 1971. [1] Though VX has been identified in several parts of the body, extraoral VX was first described by Santa Cruz and Martin, who reported two vulval lesions. [2] Most frequently encountered sites for oral lesion are gingiva, alveolus, and hard palate. [3]

VXs are usually asymptomatic, solitary; though multifocal lesions have also been reported. They are slow-growing lesions; normal, red, or yellowish in color with sessile or pedunculated, papillary, granular or verrucous appearance. They rarely reach more than 1 cm in diameter. [4] Males are affected slightly more frequently than females. The majority of cases have been reported in Caucasians. [5]

Clinically VX needs to be differentiated from papilloma, verruca, verrucous carcinoma, and, sometimes, squamous cell carcinoma. Diagnosis is made on the basis of histological appearance of the lesions, showing hyperplastic parakeratinzed squamous epithelium with elongated rete pegs of relatively uniform depth. [6],[7] Nowparast et al. in their series of 54 cases of VX demonstrated three different architectural appearances of this lesion as seen under light microscope: A] a warty or verrucous appearance, B] a papillary or cauliflower architecture, and C] a slightly raised or flat lesion. [8]

The outstanding feature of the lesion is the presence of large swollen 'foam cells' or 'Xanthoma cells,' which fill the connective tissue papillae between the epithelial pegs. Underlying stroma shows mixed chronic inflammatory cell infiltration. [1] Only three recurrences have been reported so far. [5],[8],[9] Excision is usually the treatment of choice. [3] However, no potential for malignancy for this lesion has been reported in the literature to date.


   Case History Top


Case 1

A 50-year-old man reported to the Institute of Dental Sciences, KLE University, Belgaum, with a chief complaint of dirty teeth. He gave a history of tobacco chewing 4 to 5 times a day since the last 15 years. Intraoral examination revealed a sessile, nonscrapable whitish yellow granular lesion measuring about 1 2 cm in diameter on the right buccal mucosa, which was nontender [Figure 1]. His oral hygiene was poor, with generalized stains and calculus. The patient was unaware of the lesion. Clinically the lesion was diagnosed as leukoplakia, and an excisional biopsy was preformed under local anesthesia.

Case 2

A 40-year-old man reported to the Institute of Dental Sciences, KLE University, Belgaum, with a chief complaint of missing teeth, for which he wanted replacement. He gave history of trauma to his lower jaw, inflicted 5 years back - later noting mobility of anterior teeth, which he reported were extracted. Intraoral examination revealed that 41, 42, 31, and 32 were missing. Two yellowish red slightly raised lesions on the lower labial mucosa near the mucogingival junction, measuring about 0.5 0.5 cm and 1 1 cm in diameter, were noted [Figure 2]. They were soft in consistency, nontender and not fixed to the underlying mucosa. The patient was unaware of the lesion. Clinically the lesion was diagnosed as hypertrophic Fordyce's spots, and an excisional biopsy was preformed under local anesthesia.

The lesions in these two cases showed similar microscopic appearance. Under low magnification, the section showed papillary projections lined by epithelium consisting of core of connective tissue [Figure 3]. Under higher magnification, the epithelium was parakeratinzed stratified squamous type with parakeratin plugging. Between the strands of epithelium, the connective tissue papillae extend up to the surface, with a thin layer of epithelium covering it. In these extensions, the stromal tissue appeared to be replaced by large cells [Figure 4]. Under higher magnification, these swollen cells showed clear-to-eosinophilic granular cytoplasm with eccentrically placed nuclei. These large swollen cells, also called 'foam cells' or 'xanthoma cells,' filled the connective tissue papillae between the epithelial pegs [Figure 5]. A mild-to-moderate degree of inflammatory cell infiltration consisting mainly of lymphocytes was observed in the stroma. Based on these histopathological features, diagnosis of verruciform xanthoma was given.


   Discussion Top


In the biological profile of oral verruciform xanthoma [VX], based on a worldwide literature survey of 282 cases, it was seen that oral lesions of VX were mostly seen in males, with a male-to-female ratio of 1.1: 1. Below the age of 50, the male-to-female ratio was 1.6:1; and this ratio reversed after the age of 50 in favor of females, when the male-to-female ratio was 0.8:1. The most common location for VX reported is masticatory mucosa [73.4%], with gingival margin accounting for 85 [49.1%] cases. Next in order of frequency were hard palate [ n = 25] [of these, 17 cases were contained in the 85 cases from gingival margin]; tongue [ n = 16, of which 8 were localized to the dorsum/lateral border]; buccal mucosa/vestibular fold [ n = 12]; floor of the mouth [ n = 10] and attached gingiva/alveolar mucosa [ n = 9]; soft palate [ n = 8]; lower lip [ n = 5]; junction between hard and soft palate [ n = 3]. [3]

In 1961, Shafer first reported 15 cases of VX of the oral mucosa. [1] Although in most cases VX tends to occur as an isolated lesion, in several cases the lesion developed in association with other diseases, including snuff dipper's keratoses, [10] oral pemphigus vulgaris, [11] carcinoma in situ, [12] regressive dystrophic epidermolysis bullosa, [13] lichen sclerosus, [2] solar keratoses, [14] discoid lupus erythematosus, [15] epithelial nevus or epidermal nevus syndrome, [6] lichen planus [16] and congenital hemidysplasia with ichthyosiform erythroderma and limb defects [CHILD syndrome]. [17]

The lesion did not seem to be associated with systemic abnormality of lipid metabolism, with the exception of one case, which was associated with systemic lipid storage disease. That case was reported as a multifocal case, involving the tongue, larynx, epiglottis, right glottis, and peritonsillar mucosa. [18]

Zegarelli et al. introduced the concept that local trauma and inflammation lead to epithelial entrapment and the cause of accumulation of lipid-containing macrophages is epithelial degeneration. The products of epithelial breakdown elicit an inflammatory response, which is manifested by a predominant neutrophilic infiltrate in the epithelium and a subsequent release of lipid material through the epithelium that finally is scavenged by the macrophages. [19] Several authors have supported this theory. Zegarelli et al. suggested a 'local irritant' as the initiator of this process. [7],[9],[12],[20]

Discussing the epithelial hyperplasia with elongation of the epithelial rete ridges, Mostafa et al. suggested that this elongation is illusory and is not a proliferation of epithelial cells with downward growth of the rete pegs, but rather results from the upward pushing effect of the accumulated macrophages towards the epithelium. This according to the authors reflects the thinning of the epithelium overlying the macrophages in the connective tissue papillae. [21]

Nowparast et al. suggested that the verrucous and papillary architecture may be secondary to the presence of foam cells, which affect the nutrition and metabolism of the epithelial cells, leading to a hyperkeratotic change. These authors suggested that the lipid material found in the xanthoma cells could be of the same nature as that found within foam cells in chronic inflammatory reactions such as dental granulomas and periapical cysts, where epithelial degeneration is common. [8],[22] Travis et al. also supported the view that the xanthoma cells are macrophages responsible for removal of lipid that accumulates in the submucosal tissues and that epithelial hyperplasia is a secondary event. Macrophages are known to produce a variety of growth factors that might play a role in inducing epithelial hyperplasia. [18]

Mostafa et al. observed that all xanthoma cells were negative for S-100 protein, which is in accordance with the results obtained by Rowden et al. S-100 protein-positive dendritic cells and Langerhans cells [LCs] have been found in the basal and suprabasal epithelium [melanocytes] by some investigators. Since both macrophages and LCs are known to be antigen-presenting cells, Mostafa et al. suggested that the migration of such a large number of macrophages towards the epithelium might be a compensatory phenomenon. [21]

Rowden et al. were of the opinion that VX belongs to a new category of 'non-X histiocytosis,' in which the presence of Langerhans cells suggested an immunological pathogenesis. [23] Mostafa et al. suggested that VX may be a local immunological disorder, most probably of cell-mediated mechanism, although the exact cause of this reaction could not be determined. Oliveira et al., in a very recent report, suggested that the pathological process of VX may be based on an immunological response similar to that of lichen planus. [21]

A viral etiology has also been speculated since extraoral lesions usually occur in the genitalia. [24] Santa Cruz and Martin also suggested that a viral cause was possible, despite the lack of intraepithelial inclusions or pronounced vacuolation; and they recommended further ultrastructural examination of the epithelium for the presence of viral particles. Although many ultrastructural studies have been pursued, viral particles have not been seen in either the epidermal or epithelial cells. [2] Recently, three studies disclosed negativity for human papilloma virus in the lesions by using in situ hybridization. [18],[24] Many histochemical, immunohistochemical, and ultrastructural studies have been done so far; but even today pathogenesis is obscure and needs to be clarified.

Thus in conclusion, it can be stated that although VX has an otherwise asymptomatic clinical course, yet histopathologically it still poses a challenge to the understanding of its pathogenesis. The two cases presented in this report had varied clinical appearance but very similar and characteristic histopathological presentation to be diagnosed as VX.

 
   References Top

1.Shafer WG. Verruciform xanthoma. Oral Surg 1971;31:784-9.  Back to cited text no. 1  [PUBMED]  
2.Santa Cruz DJ, Martin SA. Verruciform xanthoma of the vulva: Report of two cases. Am J Clin Pathol 1979;71:224-8.  Back to cited text no. 2  [PUBMED]  
3.Philipsen HP, Reichart PA, Takata T, Ogawa I. Verruciform xanthoma-biological profile of 282 oral lesions based on a literature survey with nine new cases from Japan. Oral Oncol 2003;39:325-36.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]
4.Polonowita AD, Firth NA, Rich AM. Verruciform xanthoma and concomitant lichen planus of the oral mucosa: A report of three cases. Int J Oral Maxillofac Surg 1999;28:62-6.  Back to cited text no. 4  [PUBMED]  
5.Iamaroon A, Vickers RA. Characterization of verruciform xanthoma by in situ hybridisation and immunohistochemistry. J Oral Pathol Med 1996;25:395-400.  Back to cited text no. 5  [PUBMED]  
6.Palestine RF, Winkelmann RK. Verruciform xanthoma in an epithelial nevus. Areh Dermatol 1982;118:686-91.  Back to cited text no. 6    
7.Cobb CM, Holt R, Denys FR. Ultrastructural features of the verruciform xanthoma. J Oral Pathol 1976;5:42-51.  Back to cited text no. 7  [PUBMED]  
8.Nowparast B, Howell FV, Rick GM. Verruciform xanthoma: A clinicopathologic review and report of fifty-four cases. Oral Surg Oral Med Oral Pathol 1981;51:619-25.  Back to cited text no. 8  [PUBMED]  
9.Neville B. The verruciform xanthoma: A review and report of eight new cases. Am J Dermatopathol 1986;8:247-53.  Back to cited text no. 9  [PUBMED]  
10.Neville BW, Weathers DR. Verruciform xanthoma. Oral Surg Oral Med Oral Pathol 1980;49:429-34.  Back to cited text no. 10  [PUBMED]  
11.Gehrig RD, Baughman RA, Collins JR. Verruciform xanthoma in a young male patient with a past history of pemphigus vulgaris. Oral Surg Oral Med Oral Pathol 1983;55:58-61.  Back to cited text no. 11    
12.Drummond JF, White DK, Damm DD, Cramer JR. Verruciform xanthoma within carcinoma in situ. J Oral Maxillofacial Surg 1989;47:398-400.  Back to cited text no. 12    
13.Cooper TW, Santa G, Bauer EA. Verruciform xanthoma: Occurrence in eroded skin in a patient with recessive dystrophic epidermolysis bullosa. J Am Acad Dermatol 1983;8:463-7.  Back to cited text no. 13    
14.Jensen JL, Liao SY, Jeffes EW. Verruciform xanthoma of the ear with coexisting epidermal dysplasia. Am J Dermatopathol 1992;14:426-30.  Back to cited text no. 14    
15.Meyers DC, Wooslby JT, Reddick RL. Verruciform xanthoma in association with discoid lupus erythematosus. J Cutan Pathol 1992;19:156-8.  Back to cited text no. 15    
16.Miyamoto Y, Nagayama M, Hayashi Y. Verruciform xanthoma occurring within oral lichen planus. J Oral Pathol Med 1996;25:188-91.  Back to cited text no. 16  [PUBMED]  
17.Zamora ME, Martin ML, Barat CA, Castro TA. Another child syndrome with xanthomatous pattern. Dermatologica 1990;180:263-6.  Back to cited text no. 17    
18.Travis WD, Davis GE, Tsokos M, Lebovics R, Merrick HF, Miller SP, et al. Multifocal verruciform xanthoma of the upper aerodigestive tract in a child with a systemic lipid storage disease. Am J Surg Pathol 1989;13:309-16.  Back to cited text no. 18  [PUBMED]  
19.Zegarelli DJ, Zegarelli-Schmidt EC, Zegarelli EV. Verruciform xanthoma: Further light and electron microscopic studies, with the addition of a third case. Oral Surg Oral Med Oral Pathol 1975;40:246-56.  Back to cited text no. 19  [PUBMED]  
20.Allen CM, Kapoor N. Verruciform xanthoma in a bone marrow transplant recipient. Oral Surg Oral Med Oral Pathol 1993;75:591-4.  Back to cited text no. 20  [PUBMED]  
21.Mostafa KA, Takata T, Ogawa I, Ijuhin N, Nikai H. Verruciform xanthoma of the oral mucosa: A clinicopathological study with immunohistochemical findings relating to pathogenesis. Virchows Arch A 1993;423:243-8.   Back to cited text no. 21    
22.Shear M. Cysts of the oral regions. 3 rd ed. Oxford: Butterworth-Heinemann Ltd; 1992.  Back to cited text no. 22    
23.Rowden D, Lovas G, Shafer W, Sheikh K. Langerhans cells in verruciform xanthoma: An immunoperoxidase study of 10 oral cases. J Oral Pathol 1986;15:48-53.  Back to cited text no. 23    
24.Helm KF, Hopfl RM, Kreider JW, Lookingbill DP. Verruciform xanthoma in an immuno-compromised patient: A case report and immunohistochemical study. J Cutan Pathol 1993;20:84-6.  Back to cited text no. 24    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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