Thelansis

Epidermodysplasia verruciformis (EV) is a rare, lifelong, cutaneous, autosomal recessive immune system disorder characterized by increased susceptibility to cutaneous human papillomavirus (HPV) infection beginning in childhood. The disease usually manifests during infancy (7.5% of cases), youth (61.5% of cases), or puberty (22% of cases) with the progressive development of hyperpigmented or hypopigmented flat wart-like papules, irregular reddish-brown plaques, seborrheic keratosis-like lesions and pityriasis Versicolor-like macules on the trunk, neck, face, dorsal hands, and feet. Loss-of-function mutations can cause EV in either of two adjacent genes, EVER1/TMC6 or EVER2/TMC8 (17q25.3), which code for membrane proteins that form a complex with the zinc transporter protein ZnT-1 in the ER membrane of keratinocytes. In epidermodysplasia verruciformis, two clinical presentations have been described. Flat-topped papules and plaques with a scaly surface and irregular borders that are white...