It is a complex disease with ectodermal, neurologic, ocular and musculoskeletal manifestations. Because of severe skin lesions in the neonatal period, the syndrome is better known by dermatologists than dentists.
Skin lesions appear normaly in the first weeks after birth.
They can be generalised all over the body or can be very local. The skin lesions heal after several months but leave pigmented areas on the skin, which is an important characteristic of the syndrome. In later stages of IP , atrophic and hairless streaks can be found on the body. These symptoms do not necessarily occur together, suggesting a complex genetic origin of the disease. Therefore IP is certainly underdiagnosed if the symptoms are limited to agenesis of only a few teeth and inconspicuous skin lesions.