Verruciform xanthoma in an epithelial nevus

Abstract

Verruciform xanthoma is an unusual lesion that was initially reported in 1971. Clinically, it is a verrucous, vascular, epithelial plaque or papillomatous growth commonly found on oral or mucosal tissues. Histologically, it is characterized by a verruciform epithelium with abundant parakeratosis and by foam cells occupying the vascularized dermal papillae. This is the second extramucosal verruciform xanthoma reported. Our patient had several unique features. She is the youngest patient described to date, her lesion is the largest reported, and the lesion occurred in an epithelial nevus-type eruption of a phocomelic extremity. Light and electron microscopic studies disclosed abnormal keratinization, mast cell proliferation, and the previously described foam cells. We believe that the verruciform xanthoma in our patient represents part of a reaction to the epithelial nevus occurring in association with a developmental anomaly.

The CHILD Nevus: A Distinct Skin Disorder

“…Some authors have reported cases of CHILD nevus under the term 'verruciform xanthoma' [18,41,42], It should be borne in mind, however, that this term does not refer to a clinicopathological entity but rather describes a histopathological feature that may be found in various other inflammatory skin diseases such as recessive dystrophic epidermolysis bullosa [431 or pemphigus vulgaris [44]. In particular, changes of verruciform xanthoma are often observed in biopsies taken from the oral cavity, the vulva or the penis [45][46][47][48], All this notwithstanding, when compared to other epi dermal nevi the phenomenon of verruciform xanthoma is a distinctive feature of CHILD nevus [3, 4|.…”

A Novel Somatic Mutation of the 3β-Hydroxysteroid Dehydrogenase Gene in Sporadic Cutaneous Verruciform Xanthoma

“…Verruciform xanthoma has been associated with a wide variety of disorders such as recessive dystrophic epidermolysis bullosa, 2 lymphedema, 3 graft vs host disease, and epidermal nevus. 4,5 It has also been reported in patients with no other associated abnormality. The occurrence of VX in other disorders has led to the suggestion that it is a morphologic change rather than a true entity.…”

“…Although studies have identified the foamy cells as lipid-laden macrophages, 5,14,16,18 the process by which the lipid accumulates has not been elucidated. Zegarelli et al 18 proposed that epithelial degeneration, along with loss of basal lamina, probably caused by a local irritant, is the initiating event.…”

Verruciform Xanthoma-Histopathologically: A Distinct Entity

“…6 Approximately 75% of all oral VX lesions occur on the masticatory mucosa of the palate, gingiva or alveolar ridge. In the largest series of 282 cases of oral VXs reported by Philipsen et al, the most commonly affected site was the gingiva (57.4%), followed by the tongue (10.3%), hard palate (7.1%), buccal or vestibular mucosa (6.7%), floor of the mouth (4.6%), and soft palate (3.2%).…”