T-cell immunodeficiency with epidermodysplasia verruciformis (EV) is a rare genetic disorder that affects the immune system. It is caused by a mutation in the gene that codes for the protein TAP1, which is responsible for transporting certain molecules across the cell membrane. People with this disorder have a weakened immune system, which makes them more susceptible to infections and skin conditions. The most common symptom of EV is the development of flat, wart-like lesions on the skin, which are caused by a virus called human papillomavirus (HPV). These lesions can be disfiguring and can lead to skin cancer if left untreated. Treatment typically involves antiviral medications and topical treatments to reduce the appearance of the lesions.

The symptoms of T-cell immunodeficiency with epidermodysplasia verruciformis (EV) include:

-Recurrent skin infections, such as warts, molluscum contagiosum, and herpes simplex virus
-Severely dry and scaly skin
-Skin Lesions that are flat, reddish-brown, and scaly
-Skin Lesions that are wart-like and may be itchy or painful
-Frequent skin infections that do not respond to treatment
-Frequent respiratory infections
-Delayed growth and development
-Frequent fevers
-Enlarged lymph nodes
-Enlarged liver and spleen
-Anemia
-Low white blood cell count
-Low platelet count
-Low levels of immunoglobulins

The cause of T-cell immunodeficiency with epidermodysplasia verruciformis (EV) is unknown. However, it is believed to be caused by a genetic mutation in the gene that codes for the protein, T-cell receptor gamma (TCRG). This mutation results in a decrease in the number of T-cells, which are responsible for fighting off infections. Additionally, the mutation may also cause the body to be unable to recognize and fight off certain types of viruses, such as the human papillomavirus (HPV). This can lead to the development of skin lesions, which are characteristic of EV.

The primary treatment for T-cell immunodeficiency with epidermodysplasia verruciformis is to manage the skin lesions. This can be done with topical medications such as retinoids, imiquimod, and cidofovir. Photodynamic therapy and laser treatments may also be used. Systemic medications such as acitretin, cimetidine, and interferon may be used to reduce the number of lesions.

In addition, it is important to maintain a healthy lifestyle and diet to support the immune system. This includes eating a balanced diet, getting regular exercise, and avoiding smoking and alcohol. Vaccinations may also be recommended to help protect against infections.

1. Inherited genetic mutations in the EVER1 and EVER2 genes.
2. Exposure to certain types of human papillomavirus (HPV).
3. Immunosuppressive medications or treatments.
4. HIV infection.
5. Certain types of cancer.
6. Certain autoimmune diseases.
7. Certain inherited metabolic disorders.
8. Certain inherited genetic syndromes.
9. Certain inherited immunodeficiencies.
10. Certain inherited bone marrow disorders.

There is no cure for T-cell immunodeficiency with epidermodysplasia verruciformis, but medications can be used to help manage the symptoms. These medications include topical retinoids, such as acitretin, to reduce the number of warts, and systemic immunosuppressants, such as cyclosporine, to reduce the risk of skin cancer. Additionally, phototherapy, such as UVB light therapy, can be used to reduce the number of warts.