The condition is most often seen in females. When it occurs in males, it is lethal.
Infants with IP are born with streaky, blistering areas. When the areas heal, they turn into rough bumps. Eventually, these bumps go away, but leave behind darkened skin, called hyperpigmentation. After several years, the skin returns to normal. In some adults, there may be areas of lighter colored skin (hypopigmentation).
There is no specific treatment for IP. Treatment is aimed at the individual symptoms. For example, glasses may be needed to improve vision. Medicine may be prescribed to help control seizures or muscle spasms.
How well a person does depends on the severity of central nervous system involvement and eye problems.
When to Call a Medical Professional
Call your health care provider if:
You have a family history of IP and are considering having children
Your child has symptoms of this disorder
Genetic counseling may be helpful for those with a family history of IP who are considering having children.
James WD, Berger TG, Elston DM. Genodermatoses and congenital anomalies. James WD, Berger TG, Elston DM, eds. Andrews' Diseases of the Skin: Clinical Dermatology. 12th ed. Philadelphia, PA: Elsevier; 2016:chap 27.
Kevin Berman, MD, PhD, Atlanta Center for Dermatologic Disease, Atlanta, GA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.