"Incontinentia Pigmenti" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus,
MeSH (Medical Subject Headings). Descriptors are arranged in a hierarchical structure,
which enables searching at various levels of specificity.
A genodermatosis occurring mostly in females and characterized by skin changes in three phases - vesiculobullous, verrucous papillomatous, and macular melanodermic. Hyperpigmentation is bizarre and irregular. Sixty percent of patients have abnormalities of eyes, teeth, central nervous system, and skin appendages.
| Descriptor ID |
D007184
|
| MeSH Number(s) |
C16.131.077.445 C16.131.831.580 C16.320.850.420 C17.800.621.497 C17.800.804.580 C17.800.827.420
|
| Concept/Terms |
Incontinentia Pigmenti- Incontinentia Pigmenti
- Bloch-Siemens-Sulzberger Syndrome
- Bloch-Siemens Syndrome
- Bloch-Sulzberger Syndrome
- Bloch Sulzberger Syndrome
- Syndrome, Bloch-Sulzberger
|
Below are MeSH descriptors whose meaning is more general than "Incontinentia Pigmenti".
Below are MeSH descriptors whose meaning is more specific than "Incontinentia Pigmenti".
This graph shows the total number of publications written about "Incontinentia Pigmenti" by people in this website by year, and whether "Incontinentia Pigmenti" was a major or minor topic of these publications.
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| Year | Major Topic | Minor Topic | Total |
|---|
| 1995 | 1 | 0 | 1 |
| 1996 | 2 | 0 | 2 |
| 1999 | 1 | 1 | 2 |
| 2000 | 3 | 0 | 3 |
| 2001 | 3 | 0 | 3 |
| 2005 | 1 | 0 | 1 |
| 2006 | 2 | 0 | 2 |
| 2011 | 1 | 0 | 1 |
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Below are the most recent publications written about "Incontinentia Pigmenti" by people in Profiles.
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Vesicular rash in a newborn girl. J Fam Pract. 2015 Nov; 64(11):729-31.
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Variegated silencing through epigenetic modifications of a large Xq region in a case of balanced X;2 translocation with Incontinentia Pigmenti-like phenotype. Epigenetics. 2011 Oct 01; 6(10):1242-7.
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Incontinentia pigmenti in male patients. J Am Acad Dermatol. 2006 Aug; 55(2):251-5.
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NEMO, NFkappaB signaling and incontinentia pigmenti. Curr Opin Genet Dev. 2006 Jun; 16(3):282-8.
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Unilateral acheiria and fatal primary pulmonary hypertension in a girl with incontinentia pigmenti. Am J Med Genet A. 2005 Jun 15; 135(3):302-3.
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Multiple pathogenic and benign genomic rearrangements occur at a 35 kb duplication involving the NEMO and LAGE2 genes. Hum Mol Genet. 2001 Oct 15; 10(22):2557-67.
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A recurrent deletion in the ubiquitously expressed NEMO (IKK-gamma) gene accounts for the vast majority of incontinentia pigmenti mutations. Hum Mol Genet. 2001 Sep 15; 10(19):2171-9.
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Atypical forms of incontinentia pigmenti in male individuals result from mutations of a cytosine tract in exon 10 of NEMO (IKK-gamma). Am J Hum Genet. 2001 Mar; 68(3):765-71.
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Filamin (FLN1), plexin (SEX), major palmitoylated protein p55 (MPP1), and von-Hippel Lindau binding protein (VBP1) are not involved in incontinentia pigmenti type 2. Am J Med Genet. 2000 Sep 04; 94(1):79-84.
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Genomic rearrangement in NEMO impairs NF-kappaB activation and is a cause of incontinentia pigmenti. The International Incontinentia Pigmenti (IP) Consortium. Nature. 2000 May 25; 405(6785):466-72.