Vulvar Keratoacanthoma

Vulvar Keratoacanthoma

Article
Skin Care
Women's Health
+2
Contributed byKrish Tangella MD, MBAJan 08, 2019

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

  • KA of Vulva
  • Keratoacanthoma of Vulva

What is Vulvar Keratoacanthoma? (Definition/Background Information)

  • Vulvar Keratoacanthoma (KA) is a low-grade, malignant tumor of the hair follicles of the vulva that usually affects elderly women. Some consider it to be a variant of squamous cell carcinoma (SCC) of skin
  • Prolonged exposure to the sun’s ultraviolet rays may result in skin DNA damage, and Keratoacanthoma of Vulva may occur as a result of that exposure. Other factors that may influence its growth include Muir-Torre syndrome, immunosuppression, and infection by human papilloma virus (HPV)
  • Vulvar Keratoacanthomas may appear as precise skin nodules. The lesions may ulcerate and result in scarring. The condition may also cause stress and emotional issues for the individual
  • The treatment of choice is surgical excision of the tumor. In most cases, the prognosis of Vulvar Keratoacanthoma is excellent with suitable treatment. Generally, no metastasis is observed with this condition 

Who gets Vulvar Keratoacanthoma? (Age and Sex Distribution)

  • Vulvar Keratoacanthoma is generally seen in women aged 60-70
  • The condition is generally prevalent worldwide, though dark-skinned individuals (Asians and Africans) are affected less than light-skinned individuals (Europeans and Americans)

What are the Risk Factors for Vulvar Keratoacanthoma? (Predisposing Factors)

The risk factors for Vulvar Keratoacanthoma may include:

  • Exposure to strong sun (ultraviolet radiation) for extended periods of time during the course of work or due to regular involvement in outdoor sports or activities
  • Regular use of tanning beds or tanning parlors
  • Individuals residing in geographical regions where hot, dry, desert-like climatic conditions exist
  • Muir-Torre syndrome (a genetic disorder)
  • Individuals with weak immune systems, which could be due to cancer treatment, AIDS, or those on immunosuppressant drugs after getting an organ transplant
  • HPV infection
  • Radiation therapy
  • Previous burn sites
  • Arsenic and coal tar exposure
  • Smoking and chewing of tobacco
  • Those with sensitive skin, who burn easily from the sun
  • Caucasians are more vulnerable than dark-skinned individuals

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones 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 Vulvar Keratoacanthoma? (Etiology)

  • The cause of Vulvar Keratoacanthoma is not well known. It is a tumor of the squamous epithelium of skin and is thought to be linked to squamous cell carcinoma
  • Some researchers think that it may occur due to exposure to ultraviolet light
  • Other impelling factors include human papilloma virus, trauma, and poor immunity
  • The condition is non-infectious; it does not spread sexually (or by direct physical contact) from one individual to another

What are the Signs and Symptoms of Vulvar Keratoacanthoma?

The signs and symptoms of Vulvar Keratoacanthoma include:

  • It occurs as a distinct nodule on the vulva (the area around the vagina) and is usually solitary in nature
  • The size of the lesions range can vary from 1-2 cm
  • The lesions can grow rapidly, then stabilize in size and begin to disappear
  • It may ulcerate and bleed
  • In some instances, the tumor may seem more pigmented than the surrounding skin
  • In general, keratoacanthomas are observed on sun-exposed parts that include the face, arms, and legs. Hence, a keratoacanthoma nodule on the vulva is quite rare

How is Vulvar Keratoacanthoma Diagnosed?

A diagnosis of Keratoacanthoma of Vulva is made by:

  • Complete physical examination with detailed medical history evaluation
  • Examination by a dermatologist using a dermoscopy, which is a special device to inspect the skin
  • Wood’s lamp examination: In this procedure, the healthcare provider examines the skin using ultraviolet light. It is performed to observe a change in skin pigmentation
  • Tissue biopsy: A biopsy of the tumor is performed and sent to a laboratory for a pathological examination. A 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. Examination of the biopsy under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis

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 Vulvar Keratoacanthoma?

The possible complications due to Vulvar Keratoacanthoma may include:

  • Discomfort or irritability of the affected area; there can be discomfort while walking or sitting
  • Sexual intercourse may be painful or uncomfortable, as the lesions are present on the vaginal walls
  • Long-lasting lesions can cause self-image problems, emotional stress, and psychological trauma in some individuals, which can lead to depression
  • Even though keratoacanthomas are low-grade tumors, metastasis is not generally observed. Yet, the condition may reappear after treatment

How is Vulvar Keratoacanthoma Treated?

The treatment measures for Vulvar Keratoacanthoma may include:

  • In a majority of cases, the preferred treatment option is surgical excision and removal of the entire tumor
  • The healthcare provider may choose to frequently monitor the tumor, if it is asymptomatic. A ‘wait and watch’ approach may be adopted
  • Follow-up care with routine screening and check-ups are important and encouraged

How can Vulvar Keratoacanthoma be Prevented?

The occurrence of Vulvar Keratoacanthoma may not be preventable, but the following factors could be considered to avoid its development:

  • Avoid prolonged and chronic exposure to the sun’s ultraviolet rays. If this is unavoidable (due to an occupational requirement), then safety precautions should be taken to reduce exposure to the UV rays by using sunscreen with a high sun-protection factor, wide-brimmed hats, and protective clothing
  • Be aware of the dangers of chronic sun exposure and take the necessary measures to protect yourself. Plan and adjust your work tasks to stay out of the sun during the period when the sun is the strongest
  • Avoid excessive sunbathing (particularly if you are fair-skinned), use of tanning beds, sun lamps, and chemical agents that accelerate tanning
  • Individuals who are frequently exposed to the sun or work in the sun should get their skin examined occasionally by a healthcare provider. This is crucial if they suspect or observe any changes in the skin
  • Avoid smoking and chewing of tobacco-based products
  • Treat the underlying cause for immunosuppression

What is the Prognosis of Vulvar Keratoacanthoma? (Outcomes/Resolutions)

  • The prognosis of Keratoacanthoma of Vulva is excellent after complete excision of the tumor
  • The prognosis may be dependent upon the underlying condition and health status of the individual
  • Despite treatment, these tumors can occasionally recur 

Additional and Relevant Useful Information for Vulvar Keratoacanthoma:

  • It has not been shown that dietary factors contribute to Keratoacanthoma of Vulva development
  • Cleaning the skin too harshly with strong chemicals or soaps may aggravate the condition. Avoid strong soaps and chemicals that could potentially aggravate the condition
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Krish Tangella MD, MBA picture
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Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team

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