Definition
Verruciform epidermodysplasia is a rare and inherited dermatological disease caused by a chronic human papillomavirus (HPV) infection.
This pathology occurs already during childhood and, in most cases, is transmitted in an autosomal recessive manner (ie it is necessary to inherit two altered copies of the gene from both parents); there is no predilection for sex.
Verruciform epidermodysplasia can be favored by defects of cellular immunity and gene mutations in the 17q25.3 region, which inactivate the EVER1 and EVER2 genes. These aberrations make patients suffering from some human papillomavirus serotypes (including HPV-5, 8, 17, 20) susceptible.
Verruciform epidermodysplasia has a marked potential for neoplastic degeneration; for warty lesions to develop into skin cancers (especially squamous cell carcinomas), it takes about 10-20 years.
Most common symptoms and signs *
- Skin discoloration
- Erythema
- macules
- Nodule
- papules
- plaques
- itch
- Scales on the skin
- Skin Ulcers
Further indications
The warty epidermodysplasia is characterized by the progressive appearance of polymorphic and disseminated cutaneous signs, such as:
- Flat, isolated, hyperpigmented or hypopigmented papules;
- Irregular reddish-brown plaques;
- Peeling or hyperkeratotic lesions, sometimes confluent in psoriasiform patches;
- Erythematous macules, sometimes atrophic, pigmented or achromic, similar to those of pityriasis versicolor.
These cutaneous manifestations can be caused by different subtypes of human papillomavirus (in 80% of the cases, HPV-5 and HPV-8 were found).
The evolution of verruciform epidermodysplasia is chronic and predisposes to the development of skin cancers other than melanoma (including Bowen's disease, spinocellular and basal cell carcinomas), during the fourth or fifth decade of life. These lesions manifest themselves especially on the photoexposed skin (trunk, neck, face, back of the hands and feet).
In the photo Dede Koswara "The Tree of Indonesia", whose case was documented by several television programs. The man died on 01/30/2016 due to complications related to verruciform epidermodysplasia. Copyright Jon Frank / Shutterstock.com
The diagnosis is based on the clinical and histological picture of the warty elements or tumor lesions.
At the moment, there is no definitive therapy for verruciform epidermodysplasia, but treatments based on cryotherapy, systemic retinoids, topical use of imiquimod and 5-fluorouracil, interferon alpha and photodynamic therapy with aminolevulinic acid may be indicated. Surgical excision is the treatment of choice for squamous cell carcinoma.
In general, the prognosis is favorable, as skin tumors have a slow evolution and metastases are rare.
To prevent the evolution of verruciform epidermodysplasia in skin cancer, fundamental preventive measures are to avoid exposure to the sun and resort to photoprotection.
