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Epidermodysplasia Verruciformis

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Last updated: March 2024

Also known as: Treeman syndrome, Lewandowsky-Lutz syndrome, Lewandowsky-Lutz dysplasia

What is epidermodysplasia verruciformis?

Epidermodysplasia verruciformis (EV) is a rare inherited skin condition that predisposes a person to infections caused by the human papillomavirus (HPV).

Who gets epidermodysplasia verruciformis?

EV has been observed to affect both males and females and individuals of all races. The condition typically appears in childhood or early adulthood.

What causes epidermodysplasia verruciformis?

EV is an inherited disorder. In most cases, it is inherited in an autosomal recessive manner. This means an individual must have both copies of mutated EV genes to be affected. Most individuals can carry a copy of the mutated gene but not show signs nor symptoms. However, there has been some cases of atypical inheritance reported.

‘Acquired epidermodysplasia verruciformis’ or EV-like eruptions can result from having an impaired immune system i.e. HIV infection, organ transplantation or cancer.

What does epidermodysplasia verruciformis look like?

The disease usually manifests in childhood with multiple pink or tan flat bumps (papules), flat pinkish white or brown spots that resemble pityriasis versicolor or thicker warty lesions that resemble seborrheic keratosis.

Lesions are most numerous on the face, neck and backs of the hands and feet.

How is epidermodysplasia verruciformis diagnosed?

It is diagnosed based on clinical and skin biopsy findings. HPV subtype can be detected within the cells using specialised techniques (PCR).

How is epidermodysplasia verruciformis treated?

Treatment options will vary depending on the individual and their needs.

EV is a lifelong disease. Treatment options are available for individual lesions. These may include:

  • Cryotherapy
  • Curettage and electrodessication
  • Laser ablation
  • Topical imiquimod cream
  • Topical 5-fluorouracil cream
  • Oral retinoids i.e. acitretin and isotretinoin
  • Photodynamic therapy (PDT)
  • Surgical excision – this is the treatment of choice for skin cancers

Prevention of skin cancers is important. This can be achieved by using appropriate sun protection measures and performing regular self-examinations of whole body, including scalp, hands and feet to ensure familiarity with skin.

What is the likely outcome of epidermodysplasia verruciformis?

EV has a favourable prognosis, given skin cancers appear progressively and are usually treated before they spread.

Dr Ruby Lee and Dr Heba JibrealMarch 2024
Dr Ruby Lee and Dr Heba JibrealFebruary 2020

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