Hypertrichosis lanuginosa congenita

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Classification according to ICD-10
Q84.2 Other congenital malformations of the hair
ICD-10 online (WHO version 2019)

The hypertrichosis lanuginosa congenita is a rare congenital disease with almost the entire body (generalized) concerned hypertrichosis (proliferation and overgrowth of lanugo ) and thus a form of congenital generalized hypertrichosis .

Synonyms are: X-linked congenital generalized hypertrichosis; Hypertrichosis generalized X-linked congenital; Hypertrichosis congenital generalized X-linked

The first description is from 1984 by the Mexican doctors MA Macías-Flores, D. García-Cruz and colleagues.

Spread and cause

The frequency is given as less than 1 in 1,000,000, inheritance is autosomal dominant or X-linked dominant . The cause is not yet known.

Clinical manifestations

Clinical criteria are:

  • Manifestation at birth with persistence of the fetal lanugo hair
  • Lanugo hairs 3 to 5 cm long grow all over the body with the exception of the palms of the hands, soles of the feet and mucous membranes

That is why those affected used to be referred to as "hair, dog, monkey or lion people".

In addition, there can be tooth anomalies such as hypodontia or later tooth eruption , also hearing loss or thickened eyebrows.

literature

  • JC Salas-Alanis, LD Lopez-Cepeda, A. Elizondo-Rodriguez, ME Morales-Barrera, AR Ramos-Garibay: Hypertrichosis lanuginosa congenita treated with diode laser epilation during infancy. In: Pediatric dermatology. Volume 31, number 4, 2014 Jul – Aug, pp. 529–530, doi : 10.1111 / j.1525-1470.2012.01881.x , PMID 23025656 .
  • V. Mendiratta, B. Harjai, T. Gupta: Hypertrichosis lanuginosa congenita. In: Pediatric dermatology. Volume 25, Number 4, 2008 Jul-Aug, pp. 483-484, doi : 10.1111 / j.1525-1470.2008.00716.x , PMID 18789097 .
  • CM Littler: Laser hair removal in a patient with hypertrichosis lanuginosa congenita. In: Dermatologic surgery: official publication for American Society for Dermatologic Surgery [et al.]. Volume 23, Number 8, August 1997, pp. 705-707, PMID 9256919 .

Individual evidence

  1. ↑ a b c Encyclopedia Dermatology
  2. ↑ a b c Hypertrichosis lanuginosa, congenital. In: Orphanet (Rare Disease Database).
  3. ^ A b MA Macías-Flores, D. García-Cruz, H. Rivera, M. Escobar-Luján, A. Melendrez-Vega, D. Rivas-Campos, F. Rodríguez-Collazo, I. Moreno-Arellano, JM Cantú : A new form of hypertrichosis inherited as an X-linked dominant trait. In: Human Genetics. Volume 66, Number 1, 1984, pp. 66-70, doi : 10.1007 / bf00275189 , PMID 6698556 .
  4. ↑ Hypertrichosis lanuginosa congenita.  In: Online Mendelian Inheritance in Man . (English)
  5. ^ Rare Diseases

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