Verruciform Xanthoma Clinical Presentation

Updated: Nov 07, 2019
  • Author: W Clark Lambert, MD, PhD; Chief Editor: William D James, MD  more...
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Presentation

History

Patients usually present with a history of an asymptomatic or tender lesion on the skin or mucosa.

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Physical Examination

The clinical appearance of a verruciform xanthoma is not diagnostic; the diagnosis is almost always made at histologic examination. Depending on the nature of the individual lesion, verruciform xanthoma may clinically resemble any verrucous, papillary, or lichenoid oral lesion, particularly any such lesion that is also hyperkeratotic. It is frequently misdiagnosed at clinical examination as a papilloma.

The lesion may appear as a verrucous, papillary, or flat-to-lichenoid process varying from 0.2-2 cm in diameter, although one lesion 4 cm in diameter has been reported. A giant verruciform xanthoma measuring 15 cm x 20 cm was reported in a 54-year old man with developmental delay. [78] Depending on the degree of keratinization, the xanthoma may appear gray to reddish pink. The process may be pedunculated but usually is sessile. At least two cases occurring in cysts have been reported.

Although almost any part of the mouth may be involved, the alveolar ridge, hard palate, and gingiva have been noted as preferential sites. [79] Because of this predilection, inflammation caused by mild trauma has been proposed to play a role in the etiopathogenesis. [28, 41] A second hypothesis suggests that the verrucous and papillary epithelial architecture may be secondary to the presence of foamy cells, which affect the metabolism of the epithelial cells, leading to hyperkeratotic change. [28, 80]

The lesions that occur on the oral mucosa or scrotum are usually not associated with any predisposing disorder.

The lesions that occur on the feet are often associated with lymphedema.

The skin lesions that occur outside of the perineum or those that are multiple are often associated with another disease process.

Examples include several cases of epidermal nevi and congenital hemidysplasia with ichthyosiform erythroderma and limb defect (CHILD) syndrome, [81, 82] in which verruciform xanthomata may be the only ostensible sign, [83] and one case each of chronic eczema with ichthyosis, chronic severe sun damage, recessive dystrophic epidermolysis bullosa, [84] and discoid lupus erythematosus. [85] Lesions have also been found in the oral cavity in association with lichen planus, [59, 86] pemphigus vulgaris, [87] oral bullae, carcinoma in situ, [88] or frank squamous cell carcinoma [46, 89] ; the lesions were also found in a bone marrow transplant recipient. [90]

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Complications

Verruciform xanthoma can be easily cured by surgical excision without complications. However, if an underlying disease is present, such as the CHILD syndrome (see above), this must be managed.  Two cases of verruciform xanthoma associated with squamous cell carcinoma have been reported, so careful scrutiny of the histopathology for associated keratinocyte atypia is warranted, especially in lesions associated with penile phimosis. [1, 46]

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