Epidermodysplasia Verruciformis
A rare inherited susceptibility to widespread HPV skin growths
Quick Facts
- Type: Rare inherited skin disorder
- Cause: Genetic susceptibility to certain HPV types
- Hallmark: Widespread wart-like and flat lesions
- Key concern: Increased skin cancer risk
Overview
Epidermodysplasia verruciformis (EV) is a very rare inherited skin disorder in which the immune system cannot properly control infection with certain strains of the human papillomavirus (HPV). As a result, people with EV develop widespread, persistent wart-like and flat, scaly skin growths, often beginning in childhood. The condition is sometimes dramatically described in the media as 'tree-man syndrome' because of the bark-like appearance the growths can take in severe cases.
EV is not contagious in the way ordinary warts are; rather, affected people have a specific, genetically determined vulnerability to HPV types that usually cause no trouble in others. A major concern is that some of these growths, especially on sun-exposed skin, can develop into skin cancer over time, so lifelong monitoring and sun protection are important.
Symptoms
Skin findings usually begin in childhood and persist. They can include:
- Numerous flat, slightly raised, wart-like growths, often on the hands, arms, face, and neck
- Reddish-brown or skin-colored scaly patches that can resemble certain other skin conditions
- Growths that may merge into larger plaques over time
- In severe cases, thick, horn-like or bark-like growths on the hands and feet
- Lesions that worsen with sun exposure
Over years, especially in adulthood, some growths on sun-exposed areas may change in a way that signals skin cancer, such as thickening, ulceration, or bleeding.
Causes
EV is caused by inherited changes in genes that normally help the immune system control specific HPV types. Because of these gene changes, the body cannot clear or suppress these viruses in the skin, allowing them to cause persistent growths.
- Genetic susceptibility: The inherited form usually follows an autosomal recessive pattern, meaning a child inherits an altered gene from each parent.
- Specific HPV types: Strains that are harmless in most people cause widespread lesions in those with EV.
- Acquired forms: A similar pattern can occur in people with severely weakened immunity, such as after organ transplantation or with advanced HIV infection.
Risk Factors
- Inheriting the gene changes responsible for EV, often when parents are carriers
- Parents who are related, which increases the chance of inheriting two altered copies
- A severely weakened immune system, which can cause an acquired EV-like condition
- Cumulative sun exposure, which raises the risk that lesions turn cancerous
Diagnosis
Diagnosis is based on the characteristic widespread skin lesions and supporting tests:
- Skin examination: Recognizing the pattern of widespread flat warts and scaly patches.
- Skin biopsy: Examining a sample under the microscope, which shows changes typical of EV.
- HPV testing: Identifying the specific virus types involved.
- Genetic testing: Can confirm the inherited gene changes and assist family counseling.
Treatment
There is no cure for EV, so treatment focuses on managing the skin growths, protecting against sun damage, and watching closely for skin cancer.
- Sun protection: Rigorous use of sunscreen, protective clothing, and sun avoidance, since ultraviolet light promotes cancer in these lesions.
- Topical and procedural treatments: Creams, cryotherapy, or other treatments to reduce bothersome growths, though lesions often recur.
- Removing suspicious lesions: Surgery to remove growths that show signs of turning into skin cancer.
- Regular skin checks: Lifelong monitoring by a dermatologist to catch cancers early.
Because the condition is lifelong and carries a cancer risk, ongoing specialist care is essential.
Prevention
- Practice strict sun protection from an early age to lower skin cancer risk
- Attend regular dermatology checkups for early detection of cancerous changes
- Report any growth that thickens, bleeds, or develops a sore that does not heal
- Seek genetic counseling for affected families to understand inheritance
EV itself cannot be prevented because it is inherited, but its most serious complication, skin cancer, can often be caught early with vigilant care.
When to See a Doctor
See a dermatologist if a child or adult develops widespread, persistent wart-like or scaly growths, particularly with a family history of similar skin problems. If EV is already diagnosed, seek prompt care for any lesion that:
- Grows quickly or thickens
- Bleeds or forms a sore that does not heal
- Becomes painful or changes in color
These changes can signal skin cancer, which is most treatable when found early.
Frequently Asked Questions
Is epidermodysplasia verruciformis contagious?
Not in the usual sense. People with EV have an inherited inability to control certain HPV types, so they develop widespread growths from viruses that are harmless to most people. The condition itself, and the genetic vulnerability behind it, are not caught from someone else.
Why is EV linked to skin cancer?
The persistent HPV-driven growths in EV, especially on sun-exposed skin, can develop into squamous cell skin cancer over time. Ultraviolet light increases this risk, which is why strict sun protection and regular skin checks are central to care.
What is 'tree-man syndrome'?
This is a media nickname for severe EV, in which the wart-like growths become thick and bark-like, particularly on the hands and feet. It describes an extreme presentation of the same underlying condition.
How is EV inherited?
The inherited form usually follows an autosomal recessive pattern, meaning a child inherits an altered gene from each parent. A similar acquired condition can develop in people whose immune systems are severely weakened, such as after a transplant.
Can EV be cured?
There is no cure. Management focuses on sun protection, treating bothersome growths, removing lesions that show signs of cancer, and lifelong dermatology monitoring to catch skin cancers early.
References
- MedlinePlus, U.S. National Library of Medicine, Genetics Home Reference. Epidermodysplasia verruciformis.
- National Organization for Rare Disorders (NORD). Epidermodysplasia Verruciformis.
- American Academy of Dermatology. Warts and HPV-related skin conditions.
- National Cancer Institute (NCI). Skin Cancer.