Epidermodisplasia verruciforme asociada a linfopenia idiopática de CD4+

Epidermodysplasia verruciformis (EV) is an autosomal recessive disease of the skin commonly associated with EVER1 and EVER 2 mutations, and is characterised by high susceptibility to infections associated with certain types of human papillomavirus called EV-PVH. Patients have warty lesions on the skin of varying characteristics and are often associated with skin cancer, with a strong association being found with EVER1 and EVER2 mutation gene. The case presented below concerns an absolute CD4 + lymphopenia, and establishes the hypothesis of a possible mutation of the RHOH gene as its origin.