Clinically visible hyperkeratotic papules and plaques already appear at birth or shortly thereafter, while neurological symptoms such as epilepsy and mental retardation manifest themselves during childhood. Papular epidermal nevus with “skyline” basal cell layer (PENS) is a very rare type of keratinocytic nevus and is associated with extracutaneous findings such as neurological symptoms in about 50% of the cases. Therefore, it is also referred to as PENS syndrome.
THE CASE Our 14-year-old patient showed typical signs of PENS syndrome. Not only were numerous hyperkeratotic papules and plaques present and confirmed by skin biopsies but the patient also showed some typical neurological symptoms At the first consultation: Aged 2 years, showed hyperkeratotic papules and plaques on the skin, which were already present at birth as well as various neurological symptoms such as mild mental…
