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CIM11 LD27.00

CIM-11 MMS (diagnostics, nouvelle classification OMS) — fichier du 2026-09-11

Incontinentia pigmenti

chapitre
20 Developmental anomalies
bloc
Multiple developmental anomalies or syndromes
definition
Incontinentia pigmenti is an X-linked dominant gene disorder due to abnormalities of the NF-kappa-B (NEMO) gene on chromosome Xq28. It is lethal in male fetuses but the presence of a normal second X chromosome in females results in a mosaicism which is compatible with life. Affected females present
type
category
navigateur oms (fr)
https://icd.who.int/browse/2026-01/mms/fr#1542530268 ↗