Preferred Label : incontinentia pigmenti;
MeSH definition : 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.;
MeSH synonym : bloch sulzberger syndrome; syndrome, bloch-sulzberger; bloch-sulzberger syndrome; Bloch-Siemens syndrome; Bloch-Siemens-Sulzberger syndrome;
MeSH annotation : multiple skin & other abnorm; do not confuse with INCONTINENTIA PIGMENTI ACHROMIANS
see PIGMENTATION DISORDERS;
Wikipedia link : https://en.wikipedia.org/wiki/Incontinentia pigmenti;
Origin ID : D007184;
UMLS CUI : C0021171;
- Allowable qualifiers
- Automatic exact mappings (from CISMeF team)
- DO Cross reference
- Manual NTBT mappings (CISMeF)
- Ne pas confondre avec
- ORDO relation(s)
- Record concept(s)
- Related MeSH Supplementary Concept(s)
- Semantic type(s)
- UMLS correspondences (same concept)
- Validated automatic mappings to BTNT
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.
https://www.has-sante.fr/jcms/p_3112337/fr/incontinentia-pigmenti
2019
false
false
false
France
incontinentia pigmenti
incontinentia pigmenti
signs and symptoms
case management
critical pathways
rare diseases
disabled persons
guideline
incontinentia pigmenti
---
http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=FR&Expert=464
2013
true
France
French
incontinentia pigmenti
scientific and technical information
---
http://www.dermis.net/dermisroot/fr/41915/diagnose.htm
Germany
French
diagnosis, differential
incontinentia pigmenti
image
---
http://www.dermis.net/dermisroot/fr/41763/diagnose.htm
Germany
French
incontinentia pigmenti
diagnosis, differential
image
---
https://anpgm.fr/media/documents/ANPGM_068_Incontinentia_Pigmenti_type_2_NEMO.doc
2009
France
guideline
incontinentia pigmenti syndrome
incontinentia pigmenti
Incontinentia pigmenti
---
http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=FR&Expert=435
2008
false
true
false
France
child
Ito hypomelanosis
scientific and technical information
incontinentia pigmenti
---
https://rmlg.uliege.be/article/1753
2008
Belgium
French
journal article
neurofibromatoses
tuberous sclerosis
incontinentia pigmenti
neural crest
---
https://www.therapeutique-dermatologique.org/spip.php?article1165
2008
France
scientific and technical information
incontinentia pigmenti, familial male-lethal type
incontinentia pigmenti, familial male-lethal type
incontinentia pigmenti
---