Preferred Label : Marie Unna congenital hypotrichosis;
ICD-11 definition : Marie Unna hereditary hypotrichosis is a rare isolated anomaly of the hair that may
be normal, sparse or absent at birth. When present it remains fine and sparse in the
first few years of life, then typically during the third year of life it becomes coarse
and twisted irregularly. When the hair is absent at birth it may regrow at this stage.
After puberty, progressive loss begins at the vertex and produces a severe scarring
atrophy.;
ICD-11 synonym : HYPT4 - [Hypotrichosis ] (MIM 146550); Marie Unna hypotrichosis; Hypotrichosis 4 (MIM 146550); HYPT4 - [Hypotrichosis ]; Hypotrichosis 4;
ICD-11 acronym : HYPT4;
Origin ID : 716463640;
Automatic exact mappings (from CISMeF team)
Marie Unna hereditary hypotrichosis is a rare isolated anomaly of the hair that may
be normal, sparse or absent at birth. When present it remains fine and sparse in the
first few years of life, then typically during the third year of life it becomes coarse
and twisted irregularly. When the hair is absent at birth it may regrow at this stage.
After puberty, progressive loss begins at the vertex and produces a severe scarring
atrophy.
