Congenital generalized hypertrichosis
| Classification according to ICD-10 | |
|---|---|
| Q84.2 | Other congenital malformations of the hair |
| ICD-10 online (WHO version 2019) | |
The congenital generalized hypertrichosis is a rare congenital disease with almost the entire body (generalized) concerned hypertrichosis (proliferation and overgrowth of hair ).
The synonyms used are: English Congenital hypertrichosis lanuginosa; CHL; congenital hypertrichosis universalis; hypertrichosis universalis; hypertrichosis lanuginosa; hypertrichosis lanuginosa universalis
The first description of a family with hypertrichosis comes from Ulisse Aldrovandi in 1648 , see Pedro Gonsalvus .
Occurrence
Congenital generalized hypertrichosis can occur in various diseases:
- Acanthosis nigricans
- Cornelia de Lange Syndrome
- Hereditary gingival fibromatosis
- Hypertrichosis lanuginosa congenita
- Prepuberal hypertrichosis
- Ambras syndrome (hypertrichosis universalis congenita)
distribution
The frequency is given as less than 1 in 1,000,000. Inheritance and causes are unknown or inconsistent.
Clinical manifestations
All forms can be seen at birth .
literature
- J. Frank, P. Poblete-Gutiérrez, K. Giehl: Genetically determined hair diseases. An update. In: The dermatologist; Journal of Dermatology, Venereology, and Allied Fields. Volume 64, Number 11, November 2013, pp. 830-842, doi: 10.1007 / s00105-013-2578-1 , PMID 24177665 (review).
- RM Trüeb: Hypertrichosis. In: Dermatologist Vol. 59, 2008, pp. 325–338
