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
Persons with Disabilities
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
---