What are the other Names for this Condition? (Also known as/Synonyms)

• VX (Verruciform Xanthoma)

What is Verruciform Xanthoma? (Definition/Background Information)

• Verruciform Xanthoma (VX) is an extremely rare condition that is characterized by the formation of a wart-like lesion, particularly within the oral cavity (mouth). The abnormal lesions are known as xanthomas
• The exact cause of the condition is not well-established; although, it is occasionally associated with conditions such as CHILD syndrome and epidermal nevus
• Verruciform Xanthoma is generally observed in middle-aged and elderly adults. Besides the mouth, the other sites include the scrotum, anus, vulva, and extremities
• The lesions are painless and benign. The skin lesions may present cosmetic issues and oral xanthomas may give rise to eating and chewing difficulties
• In a majority of cases, the treatment of Verruciform Xanthoma involves surgery (removal of the lesion). With appropriate surgical treatment, the prognosis of the condition is good

Who gets Verruciform Xanthoma? (Age and Sex Distribution)

• Verruciform Xanthoma is a very uncommon condition that is observed in adults (mean age 40-60 years)
• Only 1 in 10,000-40,000 individuals are known to develop Verruciform Xanthoma
• In very rare cases when associated with a genetic disorder, it may be observed in children below the age of 10 years
• Both males and females may be affected, although a slight male preference is noted
• Most cases are observed in Caucasians, although rarely African-Americans are also affected
• When lesions are present on the genitalia (mainly on the scrotum), Japanese middle-aged and older adult males are reported to be predominantly affected

What are the Risk Factors for Verruciform Xanthoma? (Predisposing Factors)

The following risk factors have been associated with Verruciform Xanthoma:

• Epidermal nevus
• Lymphedema
• Congenital Hemidysplasia with Ichthyosiform erythroderma and Limb Defects (CHILD) syndrome

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Verruciform Xanthoma? (Etiology)

• The exact cause of development of Verruciform Xanthoma is unknown. It may be associated with certain skin conditions or genetic disorders
• The condition is characterized by the presence of fatty and foamy histiocytes in the lesions

What are the Signs and Symptoms of Verruciform Xanthoma?

The signs and symptoms of Verruciform Xanthoma may include:

• Presence of benign wart-like (verruciform) lesions or plaques
• Plaques are broad, raised areas on the skin; they are usually broader than they are high
• The lesions are predominantly solitary and range in size from 2 mm to 2 cm
• Some lesions can also be polyp-shaped, sessile (narrowly attached at the base), or outwardly-projecting (papillomatous)
• Occasionally, cystic (fluid-filled) lesions are noted
• A vast majority of the lesions are present in the oral mucosa, inside the mouth or on the lips. Some have been identified as affecting the esophagus 
• The common sites within the mouth include the gums and gum margins
• When present outside the mouth (extra-oral lesions), the skin sites include the perineum (region between the anus and genitals), hands and feet, face, etc.
• Individuals with skin lesions may have an associated condition such as lymphedema or epidermal nevus
• In children with an associated genetic condition, the lesions may even affect the internal organs
• The lesions are usually painless, but can cause discomfort when it is present in the mouth, genitals, or anal region

Additional signs and symptoms of any underlying condition may be noted.

How is Verruciform Xanthoma Diagnosed?

A diagnosis of Verruciform Xanthoma may involve the following:

• Complete physical examination and a thorough medical history evaluation
• Dermoscopy: It is a diagnostic tool where a dermatologist examines the skin using a special magnified lens
• Wood’s lamp examination: In this procedure, the healthcare provider examines the skin using ultraviolet light. It is performed to examine the change in skin pigmentation
• Skin biopsy: A tissue biopsy is performed and sent to a laboratory for a pathological examination. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis

Note: A biopsy may be performed to rule out other skin conditions with similar signs and symptoms.

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Verruciform Xanthoma?

The complications of Verruciform Xanthoma may include:

• Eating and chewing difficulties
• Cosmetic concerns and emotional stress
• Scratching the lesions may lead to bleeding and ulceration, which may result in secondary infections
• This may give rise to scar formation on healing
• Complications that arise from any underlying condition

How is Verruciform Xanthoma Treated?

• In many cases, local surgical excision is performed for Verruciform Xanthoma
• The administration of medications, use of chemotherapy, or radiation therapy is not required
• The treatment of underlying associated condition may be necessary, if any present

How can Verruciform Xanthoma be Prevented?

Currently, there are no known methods to prevent the occurrence of Verruciform Xanthoma.

What is the Prognosis of Verruciform Xanthoma? (Outcomes/Resolutions)

• The prognosis of Verruciform Xanthoma is good with appropriate treatment (surgery)
• In a majority of the individuals, the lesions are not known to recur following treatment

Additional and Relevant Useful Information for Verruciform Xanthoma:

• Cleaning the skin too hard with strong chemicals or soaps may aggravate the skin condition. Care must be taken avoid strong soaps and chemicals that could potentially worsen the condition
• The presence of dirt on the body is not a causative factor for the condition. However, it helps to be clean and hygienic, which may help the condition from getting worse