Congenital Hypertrichosis Lanuginosa Treatment & Management
- Author: Sarah K Taylor, MD; Chief Editor: William D James, MD more...
Medical Care
- The use of eflornithine (Vaniqa cream), 13.9% or hair removal by means of repeated shaving, depilatory methods (eg, chemical, electric methods), or bleaching can improve congenital hypertrichosis lanuginosa (CHL) patients’ appearance.
- Older techniques of hair removal are mentioned in the literature; these rarely used techniques include diathermy and radiation therapy.
- Clinicians should consider the use of antidepressant medications in patients with psychological sequelae, including depression.
Surgical Care
- Laser hair removal has been proposed as a treatment option, although there are conflicting reports regarding the proposed efficacy of lasers in removing the vellus hairs in congenital hypertrichosis lanuginosa.
- Littler found a 40-80% reduction in unwanted hair using the Q-switched Nd:YAG laser after the application of a topical carbon-based solution. This therapy may be a well-tolerated method of hair removal in children because the lower fluences required resulted in decreased associated pain during treatment.
- Vashi et al suggest that because of the difference in the penetrance of laser light into nonpigmented hair versus pigmented hair, laser therapy may not be effective in unpigmented lanugo or vellus hairs .[20]
Consultations
- Genetic consultation may be indicated for the family members of patients with congenital hypertrichosis or Ambras syndrome.
- Congenital hypertrichosis lanuginosa and Ambras syndrome may have an autosomal dominant pattern of inheritance; however, an association with a genetic defect has not been demonstrated in all patients.
- Belengeanu et al describe 2 siblings with purported Ambras syndrome born to normal parents and propose that these patients might represent either an autosomal recessive pattern or germline mosaicism.[38]
- Psychiatric evaluation may be indicated in patients in whom the physical findings of the syndrome cause psychological morbidity.
Brandt A. Uber die sogenannten Hundemenschen, beziehungsweise uber Hypertrichosis universalis. Biol Zbl. 1897;17:161-79.
Felgenhauer WR. [Hypertrichosis languinosa universalis]. J Genet Hum. May 1969;17(1):1-44. [Medline].
Baumeister FA, Egger J, Schildhauer MT, Stengel-Rutkowski S. Ambras syndrome: delineation of a unique hypertrichosis universalis congenita and association with a balanced pericentric inversion (8) (p11.2; q22). Clin Genet. Sep 1993;44(3):121-8. [Medline].
Beighton P. Congenital hypertrichosis lanuginosa. Arch Dermatol. Jun 1970;101(6):669-72. [Medline]. [Full Text].
BROSTER LR. Hypertrichosis: a report of three cases. Br Med J. May 20 1950;1(4663):1171-4. [Medline]. [Full Text].
Cantu JM, Garcia-Cruz D, Sanchez-Corona J, Hernandez A, Nazara Z. A distinct osteochondrodysplasia with hypertrichosis- Individualization of a probable autosomal recessive entity. Hum Genet. 1982;60(1):36-41. [Medline].
Demikova NS, Blinnikova OE, Udler EE. [A case of congenital generalized hypertrichosis]. Klin Med (Mosk). Mar 1986;64(3):125-6. [Medline].
Freire-Maia N, Felizali J, de Figueiredo AC, Opitz JM, Parreira M, Maia NA. Hypertrichosis lanuginosa in a mother and son. Clin Genet. Nov 1976;10(5):303-6. [Medline].
Gardner AL. A case of hypertrichosis universalis. East Afr Med J. Jul 1964;41:345-6. [Medline].
Jalili IK. Cone-rod congenital amaurosis associated with congenital hypertrichosis: an autosomal recessive condition. J Med Genet. Aug 1989;26(8):504-10. [Medline]. [Full Text].
Janssen TAE, De Lange C. Familial congenital hypertrichosis totalis (trichostasis). Acta Paediatr. 1945;33:69-78.
Joest HR. Haarmenschen Ram-a-Samy. Z f Ethnologie. 1984;26:433-5.
Judge MR, Khaw PT, Rice NS, Christopher A, Holmstrom G, Harper JI. Congenital hypertrichosis lanuginosa and congenital glaucoma. Br J Dermatol. May 1991;124(5):495-7. [Medline].
Kint AH, Vermander FR, Decroix JM. [Congenital hypertrichosis lanuginosa]. Hautarzt. Jul 1985;36(7):423-4. [Medline].
Li ZH, Tong ZH, Luo GY, Cai HM. Congenital hypertrichosis universalis associated with gingival hyperplasia and macromastia. Chin Med J (Engl). Nov 1986;99(11):916-7. [Medline].
McKusick VA. Mendelian Inheritance in Man: Catalogs of autosomal dominant, autosomal recessive and X-linked phenotypes. 10th ed. Baltimore: John Hopkins University Press; 1992.
Nowakowski TK, Scholz A. [The fate of people with hypertrichosis throughout history]. Hautarzt. Nov 1977;28(11):593-9. [Medline].
Partridge JW. Congenital hypertrichosis lanuginosa: neonatal shaving. Arch Dis Child. Jun 1987;62(6):623-5. [Medline].
Suskind R, Esterly NB. Congenital hypertrichosis universalis. Birth Defects Orig Artic Ser. Jun 1971;7(8):103-6. [Medline].
Vashi RA, Mancini AJ, Paller AS. Primary generalized and localized hypertrichosis in children. Arch Dermatol. Jul 2001;137(7):877-84. [Medline].
Chanukvadze D, Kristesashvili J. Effectiveness of different diagnostic methods for assessment of hyperandrogenism in young women with hirsutism. Georgian Med News. Nov 2011;25-9. [Medline].
Hizli D, Kösüs A, Kösüs N, Kamalak Z, Ak D, Turhan NO. The impact of birth weight and maternal history on acne, hirsutism, and menstrual disorder symptoms in Turkish adolescent girls. Endocrine. Dec 16 2011;[Medline].
Escobar-Morreale HF, Carmina E, Dewailly D, Gambineri A, Kelestimur F, Moghetti P, et al. Epidemiology, diagnosis and management of hirsutism: a consensus statement by the Androgen Excess and Polycystic Ovary Syndrome Society. Hum Reprod Update. Nov 6 2011;[Medline].
Danforth CH. Studies on hair with special reference to hypertrichosis. Arch Dermatol Syphilol. 1925;12:380-401.
Rook A, Ebling FJG, Champion RH, Burton JL, eds. Textbook of Dermatology. Vol 3. 4th ed. Oxford and Edinburgh: Blackwell Scientific Publications; 1986.
von Luschan F. Ein Haarmensch. Zeitschrift fur Ethnologie. 1907;39:425-9.
Torbus O, Sliwa F. [Ambras syndrome--a form of generalised congenital hypertrichosis]. Pol Merkur Lekarski. Mar 2002;12(69):238-40. [Medline].
Baumeister FA, Schwarz HP, Stengel-Rutkowski S. Childhood hypertrichosis: diagnosis and management. Arch Dis Child. May 1995;72(5):457-9. [Medline].
Franklin DL, Roberts GJ. Delayed tooth eruption in congenital hypertrichosis lanuginosa. Pediatr Dent. May-Jun 1998;20(3):192-4. [Medline].
Larregue M, Vabre P, Cavaroc Y, Duriez P, Matard B. [Diffuse hamartoma of the arrector muscles and congenital hypertrichosis lanuginosa]. Ann Dermatol Venereol. 1991;118(11):796-8. [Medline].
Verloes A, Massin M, Fransolet AC, Misson JP. Hypertrichosis, Fallot tetralogy, growth and developmental delay. Clin Dysmorphol. Oct 2004;13(4):247-50. [Medline].
Mendiratta V, Harjai B, Gupta T. Hypertrichosis lanuginosa congenita. Pediatr Dermatol. Jul-Aug 2008;25(4):483-4. [Medline]. [Full Text].
Balducci R, Toscano V, Tedeschi B, et al. A new case of Ambras syndrome associated with a paracentric inversion (8) (q12; q22). Clin Genet. Jun 1998;53(6):466-8. [Medline].
Tadin M, Braverman E, Cianfarani S, et al. Complex cytogenetic rearrangement of chromosome 8q in a case of Ambras syndrome. Am J Med Genet. Jul 22 2001;102(1):100-4. [Medline].
Tadin-Strapps M, Warburton D, Baumeister FA, et al. Cloning of the breakpoints of a de novo inversion of chromosome 8, inv (8)(p11.2q23.1) in a patient with Ambras syndrome. Cytogenet Genome Res. 2004;107(1-2):68-76. [Medline].
Stengel-Rutkowski S, Murken JD, Pilar V, et al. New chromosomal dysmorphic syndromes. 3. Partial trisomy 3q. Eur J Pediatr. Feb 8 1979;130(2):111-25. [Medline].
Martinez Santana S, Perez Alvarez F, Frias JL, Martinez-Frias ML. Hypertrichosis, atrophic skin, ectropion, and macrostomia (Barber-Say syndrome): report of a new case. Am J Med Genet. Aug 1 1993;47(1):20-3. [Medline].
Belengeanu V, Rozsnyai K, Gug C, Banateanu M, Farcas S, Belengeanu A. Ambras syndrome: report on two affected siblings with no prior family history. Clin Dysmorphol. Oct 2004;13(4):265-7. [Medline].
Baumeister FA, Stengel-Rutkowski S. Differentiation of congenital hypertrichosis from Ambras syndrome. Clin Genet. Dec 1994;46(6):441. [Medline].
Bondeson J, Miles AE. Julia Pastrana, the nondescript: an example of congenital, generalized hypertrichosis terminalis with gingival hyperplasia. Am J Med Genet. Aug 15 1993;47(2):198-212. [Medline].
Bondeson J, Miles AE. The hairy family of Burma: a four generation pedigree of congenital hypertrichosis lanuginosa. J R Soc Med. Jul 1996;89(7):403-8. [Medline].
Fred FF. Differential diagnosis of excess hair in women. In: Ferri's Clinical Advisor: Instant Diagnosis and Treatment. St Louis: Mosby; 2001:892.
Littler CM. Laser hair removal in a patient with hypertrichosis lanuginosa congenita. Dermatol Surg. Aug 1997;23(8):705-7. [Medline].
Rashid M Rashid, Lucile E. White. A hairy development in hypertrichosis: a brief review of Ambras syndrome. Dermatology Online Journal [serial online]. 2007;13 (3):8. Available at http://dermatology.cdlib.org/133/reviews/ambras/rashid.html.
Sigalas J, Tabakis T, Skordala M, Nouri M. Congenital Hypertrichosis Universalis. Pediatrica Chronica. 1990;17:181-5.

