What is Incontinentia Pigmenti?

What is Incontinentia Pigmenti?

• Incontinentia Pigmenti: A rare genetic skin pigmentation disorder characterized by eye, teeth, bone, nail and hair malformations as well as central nervous abnormalities and mental deficiency.
• Incontinentia Pigmenti: A skin pigmentation disorder with malformations of the eyes, teeth, bones, nails, heart, central nervous system, and hair. Mental deficiency is usually associated. The syndrome is divided into two forms: Incontinentia pigmenti type I and type II which lethal in males.
  Source - Diseases Database

Incontinentia Pigmenti is listed as a "rare disease" by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH). This means that Incontinentia Pigmenti, or a subtype of Incontinentia Pigmenti, affects less than 200,000 people in the US population.
Source - National Institutes of Health (NIH)

Ophanet, a consortium of European partners, currently defines a condition rare when it affects 1 person per 2,000. They list Incontinentia Pigmenti as a "rare disease".
Source - Orphanet

Incontinentia Pigmenti: Introduction

Types of Incontinentia Pigmenti:

Broader types of Incontinentia Pigmenti:

• Genetic Disease
• Dominant Genetic Diseases
• X-linked Genetic Diseases
• X-linked Dominant Genetic Diseases
• Skin conditions
• more types...»

How many people get Incontinentia Pigmenti?

Prevalance of Incontinentia Pigmenti: 700 - 1,000 cases of incontinentia pigmenti have been reported, Genetics Home Reference website
Prevalance Rate of Incontinentia Pigmenti: approx 1 in 388,571 or 0.00% or 700 people in USA [about data]

How serious is Incontinentia Pigmenti?

Complications of Incontinentia Pigmenti: see complications of Incontinentia Pigmenti
Prognosis of Incontinentia Pigmenti: Although the skin abnormalities usually regress, and sometimes disappear completely, there may be residual neurological difficulties. (Source: excerpt from NINDS Incontinentia Pigmenti Information Page: NINDS)

What causes Incontinentia Pigmenti?

Causes of Incontinentia Pigmenti: see causes of Incontinentia Pigmenti
Causes of Incontinentia Pigmenti: The disorder is caused by excessive deposits of melanin (normal skin pigment). (Source: excerpt from NINDS Incontinentia Pigmenti Information Page: NINDS)

What are the symptoms of Incontinentia Pigmenti?

Symptoms of Incontinentia Pigmenti: see symptoms of Incontinentia Pigmenti

Complications of Incontinentia Pigmenti: see complications of Incontinentia Pigmenti

Can anyone else get Incontinentia Pigmenti?

Inheritance: see inheritance of Incontinentia Pigmenti

Inheritance of genetic conditions: see details in inheritance of genetic diseases.

Incontinentia Pigmenti: Testing

Diagnostic testing: see tests for Incontinentia Pigmenti.

Misdiagnosis: see misdiagnosis and Incontinentia Pigmenti.

How is it treated?

Treatments for Incontinentia Pigmenti: see treatments for Incontinentia Pigmenti
Research for Incontinentia Pigmenti: see research for Incontinentia Pigmenti

Organs Affected by Incontinentia Pigmenti:

Organs and body systems related to Incontinentia Pigmenti include:

• Skin

Name and Aliases of Incontinentia Pigmenti

Main name of condition: Incontinentia Pigmenti

Class of Condition for Incontinentia Pigmenti: genetic x-linked dominant

IP, Bloch-Sulzberger syndrome, Incontinentia pigmenti, familial male-lethal type, Incontinentia pigmenti, type 2, (formerly), IP2, (formerly)

Bloch-Sulzberger syndrome, Siemens-Bloch-Sulzberger melanoblastosis Source - Diseases Database

Bloch-Sulzberger syndrome, IP, IP2, (formerly), Incontinentia pigmenti, familial male-lethal type, Incontinentia pigmenti, type II, (formerly), IP2, (formerly), Incontinentia pigmenti, familial male-lethal type, Incontinentia pigmenti, type II, (formerly), Bloch-Sulzberger syndrome
Source - Office of Rare Diseases (ORD) of the National Institutes of Health (NIH)

Research the causes of these diseases that are similar to, or related to, Incontinentia Pigmenti:

• Bloch- Sulzberger disease
• Alopecia
• Hypodontia
• Wart like rash
• Somatic asymmetry
• Hemivertebrae scoliosis
• Spina bifida

 » Next page: Prevalence and Incidence of Incontinentia Pigmenti

Rate This Website

What do you think about the features of this website? Take our user survey and have your say:

Website User Survey

Medical Tools & Articles:

Next articles:

• Prevalence and Incidence of Incontinentia Pigmenti
• Videos related to Incontinentia Pigmenti
• Prognosis of Incontinentia Pigmenti
• Types of Incontinentia Pigmenti
• Causes of Incontinentia Pigmenti

Tools & Services:

• Bookmark this page
• Take a survey relating to Incontinentia Pigmenti
• Symptom Search
• Symptom Checker
• Medical Dictionary
• Give your feedback

Medical Articles:

• Disease & Treatments Search
• Misdiagnosis Center
• Full list of interesting articles

Forums & Message Boards

• Ask or answer a question at the Boards:
• I cannot get a diagnosis. Please help.
• Tell us your medical story.
• Share your misdiagnosis story.
• What is the best treatment for my condition?
• See all the Boards.

Enter your search terms Submit search form
Search The Web Search wrongdiagnosis.com

Common Health Mistakes

Symptom
Checker

Search Specialists by State and City