Genetic disorder affecting skin (various types of lesions), hair, teeth, nails, and CNS.Encyclopedia Entry for Incontinentia Pigmenti :Incontinentia pigmenti. IP is caused by an X-linked dominant genetic defect that occurs on a gene known as IKBKG. Because the gene defect occurs on the X chromosome, the condition is most often seen in females. When it occurs in males, it is usually lethal.With the skin symptoms, there are 4 stages. Infants with IP are born with streaky, blistering areas. In stage 2, when the areas heal, they turn into rough bumps. In stage 3, the bumps go away, but leave behind darkened skin, called hyperpigmentation. After several years, the skin returns to normal. In stage 4, there may be areas of lighter colored skin ( hypopigmentation ) that is thinner. IP is associated with central nervous system problems, including: Delayed development Loss of movement (paralysis) Intellectual disability Muscle spasms Seizures People with IP may also have abnormal teeth, hair loss , and vision problems.The health care provider will perform a physical exam, look at the eyes, and test muscle movement. There may be unusual patterns and blisters on the skin, as well as bone abnormalities. An eye exam may reveal cataracts , strabismus (crossed eyes), or other problems. To confirm the diagnosis, these tests may be done: Blood tests Skin biopsy CT or MRI scan of the brain.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.These resources can provide more information about IP: Incontinentia Pigmenti International Foundation -- www.ipif.org National Organization for Rare Disorders -- rarediseases.org/rare-diseases/incontinentia-pigmenti.How well a person does depends on the severity of central nervous system involvement and eye problems.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.Bloch-Sulzberger syndrome; Bloch-Siemens syndrome.Incontinentia pigmenti on the leg Incontinentia pigmenti on the leg Incontinentia pigmenti on the leg Incontinentia pigmenti on the leg.Islam MP, Roach ES. Neurocutaneous syndromes.