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Incontinentia pigmenti

Other Names for this Disease
  • Bloch-Sulzberger syndrome
  • Incontinentia pigmenti type 2 (formerly)
  • Incontinentia pigmenti, familial male-lethal type
  • IP
  • IP2 (formerly)
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Incontinentia pigmenti (IP) is a genetic condition affecting the skin and other body systems. Skin symptoms change with time, beginning with a blistering rash in infancy, followed by wart-like skin growths, which then become swirled grey or brown patches in childhood, to swirled light patches in adulthood. Other signs and symptoms may include hair loss, small or missing teeth, eye abnormalities that can lead to vision loss, and lined or pitted fingernails and toenails. Most people with incontinentia pigmenti have normal intelligence, however some have delayed development, intellectual disability, seizures, or other neurological problems. IP is caused by mutations in the IKBKG gene. It is inherited in an X-linked dominant fashion.[1]
Last updated: 4/18/2011


  1. Incontinentia pigmenti. Genetics Home Reference. 2008; Accessed 4/18/2011.
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Basic Information

  • DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.
  • Genetics Home Reference (GHR) contains information on Incontinentia pigmenti. This website is maintained by the National Library of Medicine.
  • The Incontinentia Pigmenti International Foundation Web site provides information on this topic. Click on the link above to view the information page.
  • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
  • The National Institute of Neurological Disorders and Stroke (NINDS) collects and disseminates research information related to neurological disorders. Click on the link to view information on this topic.
  • The National Organization for Rare Disorders (NORD) is a federation of more than 130 nonprofit voluntary health organizations serving people with rare disorders. Click on the link to view information on this topic.

In Depth Information

  • Medscape Reference provides information on this topic. Click on the link to view this information. You may need to register to view the medical textbook, but registration is free.
  • MeSH® (Medical Subject Headings) is a terminology tool used by the National Library of Medicine. Click on the link to view information on this topic.
  • The Online Mendelian Inheritance in Man (OMIM) is an catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs.  Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Incontinentia pigmenti. Click on the link to view a sample search on this topic.

Selected Full-Text Journal Articles