Desmoplastic giant congenital nevus with progressive depigmentation

Desmoplastic hairless hypopigmented nevus is an extremely rare sclerotic, alopecic, and progressively hypopigmented giant congenital melanocytic nevus, which is histologically characterized by an intense desmoplasia. A significant trend toward spontaneous involution has been described. We report a case of desmoplastic hairless hypopigmented nevus that underwent a progressive depigmentation associated with loss of its woody consistency. The loss of induration appears to be the main marker for the complete regression of these nevi.