The family of human papillomaviruses (HPV) is very large: there are indeed more than 200 HPV viruses that can potentially infect humans. While some can be responsible for plantar or genital warts, others can cause much more serious diseases – such as cancer of the cervix.
Epidermodysplasia verruciformis (also known as “tree-man syndrome” or “Lutz-Lewandowsky syndrome”) is a rare dermatological disease caused by an HPV viral infection: in about 80% of cases, the HPV 5 or HPV 8 viruses are responsible. To date, approximately 200 cases have been documented worldwide.
The tree-man syndrome usually appears during childhood: it is characterized by the appearance of warts and “horns” on the skin. The affected areas (mainly in the hands and feet) then look like bark – hence the name of this very debilitating disease.
The tree-man syndrome, a probably genetic disease
Beyond its aesthetic appearance, epidermodysplasia verruciformis is a serious pathology since 30% to 60% of affected patients develop skin cancer (squamous cell carcinoma) around the age of 40-50 years.
Until now, the exact origin of the tree-man syndrome was not known. But a recent study conducted by Inserm, the University of Paris and the AP-HP has just highlighted a potential genetic cause. The researchers thus noticed that the patients affected by this disease all presented a mutation of the CD28 gene: this gene plays (in normal times) a major role in the activation of T lymphocytes, these immune cells which are responsible for destroying virus-infected cells.
“In these patients, the CD28 gene mutation prevents the immune system from recognizing the virus and triggering an appropriate response. The virus then proliferates in the keratinocytes, the cells that make up the epidermis of the skin, and causes an anarchic multiplication of warts and/or cutaneous horns.“say the researchers, who published their work in the specialized journal Cell.
To date, it is not possible to cure the man-tree syndrome. A hematopoietic stem cell transplant (to replace the patient’s immune system) can be considered: however, this is a long and expensive treatment.
Source : OrphaNet
Read also :
- Anal cancer: the other cancer due to the papillomavirus
- Cervical cancer: should I vaccinate my teenager or not?
- What you need to know about the papillomavirus (HPV)
