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Alopecia, congenital(ALPC)

MedGen UID:
78581
•Concept ID:
C0265992
•
Congenital Abnormality
Synonym: Congenital alopecia X-linked
SNOMED CT: Atrichia congenita (403798006); Nevoid congenital alopecia (2965006); Congenital alopecia (2965006); Congenital atrichosis (2965006)
 
Monarch Initiative: MONDO:0010229
OMIM®: 300042

Definition

A congenital condition characterized by the absence of hair on the scalp or entire body. The lack of hair is rarely absolute and is usually accompanied by incompletely grown, lanugo-like hair. It affects males twice as much as females and a familial tendency is common. [from NCI]

Professional guidelines

PubMed

Bachmann-Bupp syndrome and treatment.
Bachmann AS, VanSickle EA, Michael J, Vipond M, Bupp CP
Dev Med Child Neurol 2024 Apr;66(4):445-455. Epub 2023 Jul 19 doi: 10.1111/dmcn.15687. PMID: 37469105Free PMC Article
Prenatal diagnosis of lanosterol synthase deficiency: Fetal ultrasound findings as a window on family genetics.
Matza Porges S, Mor-Shaked H, Shaag A, Porat S, Daum H
Eur J Med Genet 2023 Oct;66(10):104825. Epub 2023 Sep 1 doi: 10.1016/j.ejmg.2023.104825. PMID: 37659595
Diagnosis and management of alopecia in children.
Castelo-Soccio L
Pediatr Clin North Am 2014 Apr;61(2):427-42. Epub 2014 Jan 21 doi: 10.1016/j.pcl.2013.12.002. PMID: 24636654

Recent clinical studies

Etiology

Genome-wide association study in frontal fibrosing alopecia identifies four susceptibility loci including HLA-B*07:02.
Tziotzios C, Petridis C, Dand N, Ainali C, Saklatvala JR, Pullabhatla V, Onoufriadis A, Pramanik R, Baudry D, Lee SH, Wood K, Liu L, Seegobin S, Michelotti GA, Lwin SM, Christou EAA, Curtis CJ, de Rinaldis E, Saxena A, Holmes S, Harries M, Palamaras I, Cunningham F, Parkins G, Kaur M, Farrant P, McDonagh A, Messenger A, Jones J, Jolliffe V, Ali I, Ardern-Jones M, Mitchell C, Burrows N, Atkar R, Banfield C, Alexandroff A, Champagne C, Cooper HL, Vañó-Galván S, Molina-Ruiz AM, Perez NO, Patel GK, Macbeth A, Page M, Bryden A, Mowbray M, Wahie S, Armstrong K, Cooke N, Goodfield M, Man I, de Berker D, Dunnill G, Takwale A, Rao A, Siah TW, Sinclair R, Wade MS, Dlova NC, Setterfield J, Lewis F, Bhargava K, Kirkpatrick N, Estivill X, Stefanato CM, Flohr C, Spector T, Watt FM, Smith CH, Barker JN, Fenton DA, Simpson MA, McGrath JA
Nat Commun 2019 Mar 8;10(1):1150. doi: 10.1038/s41467-019-09117-w. PMID: 30850646Free PMC Article
Structural insights into ligand recognition by the lysophosphatidic acid receptor LPA(6).
Taniguchi R, Inoue A, Sayama M, Uwamizu A, Yamashita K, Hirata K, Yoshida M, Tanaka Y, Kato HE, Nakada-Nakura Y, Otani Y, Nishizawa T, Doi T, Ohwada T, Ishitani R, Aoki J, Nureki O
Nature 2017 Aug 17;548(7667):356-360. Epub 2017 Aug 9 doi: 10.1038/nature23448. PMID: 28792932
Alopecia in genetic diseases.
Calvieri S, Rossi A
G Ital Dermatol Venereol 2014 Feb;149(1):1-13. PMID: 24566562
Unraveling the molecular mechanisms of hair and nail genodermatoses.
Stratigos AJ, Baden HP
Arch Dermatol 2001 Nov;137(11):1465-71. doi: 10.1001/archderm.137.11.1465. PMID: 11708949
Heritable disorders of hair.
Birnbaum PS, Baden HP
Dermatol Clin 1987 Jan;5(1):137-53. PMID: 3549074

Diagnosis

Congenital atrichia with papular lesions.
Hajare SA, Gavali S, Mukhi J, Singh RP
Dermatol Online J 2020 Apr 15;26(4) PMID: 32621683
Congenital Hypotrichosis and Congenital Atrichia.
Al-Dawsari NA, Kaliyadan F
Skinmed 2018;16(1):27-32. Epub 2018 Feb 1 PMID: 29551108
Hair loss in infancy.
Moreno-Romero JA, Grimalt R
G Ital Dermatol Venereol 2014 Feb;149(1):55-78. PMID: 24566566
Congenital triangular alopecia.
Lacarrubba F, Micali G
BMJ Case Rep 2014 Jan 8;2014 doi: 10.1136/bcr-2013-202918. PMID: 24403390Free PMC Article
Congenital atrichia and hypotrichosis.
Bennàssar A, Ferrando J, Grimalt R
World J Pediatr 2011 May;7(2):111-7. Epub 2011 May 15 doi: 10.1007/s12519-011-0262-z. PMID: 21574026

Therapy

Clinical and trichoscopic characteristics of temporal triangular alopecia: A multicenter study.
Fernández-Crehuet P, Vaño-Galván S, Martorell-Calatayud A, Arias-Santiago S, Grimalt R, Camacho-Martínez FM
J Am Acad Dermatol 2016 Sep;75(3):634-637. doi: 10.1016/j.jaad.2016.04.053. PMID: 27543220
Leptomeningeal angiomatosis accompanied by hair follicle nevus.
Okada Y, Hamano K, Iwasaki N, Iijima S, Anno I
Childs Nerv Syst 1998 Apr-May;14(4-5):218-21. doi: 10.1007/s003810050215. PMID: 9660127
Congenital triangular alopecia.
Armstrong DK, Burrows D
Pediatr Dermatol 1996 Sep-Oct;13(5):394-6. doi: 10.1111/j.1525-1470.1996.tb00708.x. PMID: 8893240
Use of the temporary soft-tissue expander in posttraumatic alopecia.
Leighton WD, Johnson ML, Friedland JA
Plast Reconstr Surg 1986 May;77(5):737-43. doi: 10.1097/00006534-198605000-00006. PMID: 3703997

Prognosis

Deficiency of the human cysteine protease inhibitor cystatin M/E causes hypotrichosis and dry skin.
van den Bogaard EHJ, van Geel M, van Vlijmen-Willems IMJJ, Jansen PAM, Peppelman M, van Erp PEJ, Atalay S, Venselaar H, Simon MEH, Joosten M, Schalkwijk J, Zeeuwen PLJM
Genet Med 2019 Jul;21(7):1559-1567. Epub 2018 Nov 14 doi: 10.1038/s41436-018-0355-3. PMID: 30425301Free PMC Article
Clinical and trichoscopic characteristics of temporal triangular alopecia: A multicenter study.
Fernández-Crehuet P, Vaño-Galván S, Martorell-Calatayud A, Arias-Santiago S, Grimalt R, Camacho-Martínez FM
J Am Acad Dermatol 2016 Sep;75(3):634-637. doi: 10.1016/j.jaad.2016.04.053. PMID: 27543220
Resource utilization and quality of life associated with congenital ichthyoses.
Kamalpour L, Gammon B, Chen KH, Veledar E, Pavlis M, Rice ZP, Chen SC
Pediatr Dermatol 2011 Sep-Oct;28(5):512-8. Epub 2011 Sep 5 doi: 10.1111/j.1525-1470.2011.01432.x. PMID: 21895756
Molecular basis of congenital atrichia in humans and mice.
Ahmad W, Panteleyev A, Christiano AM
Cutis 1999 Oct;64(4):269-76. PMID: 10544884
Ichthyosis follicularis, alopecia, and photophobia (IFAP) syndrome: clinical and neuropathological observations in a 33-year-old man.
Keyvani K, Paulus W, Traupe H, Kiesewetter F, Cursiefen C, Huk W, Raab K, Orth U, Rauch A, Pfeiffer RA
Am J Med Genet 1998 Jul 24;78(4):371-7. doi: 10.1002/(sici)1096-8628(19980724)78:4<371::aid-ajmg13>3.0.co;2-f. PMID: 9714442

Clinical prediction guides

Genome-wide association study in frontal fibrosing alopecia identifies four susceptibility loci including HLA-B*07:02.
Tziotzios C, Petridis C, Dand N, Ainali C, Saklatvala JR, Pullabhatla V, Onoufriadis A, Pramanik R, Baudry D, Lee SH, Wood K, Liu L, Seegobin S, Michelotti GA, Lwin SM, Christou EAA, Curtis CJ, de Rinaldis E, Saxena A, Holmes S, Harries M, Palamaras I, Cunningham F, Parkins G, Kaur M, Farrant P, McDonagh A, Messenger A, Jones J, Jolliffe V, Ali I, Ardern-Jones M, Mitchell C, Burrows N, Atkar R, Banfield C, Alexandroff A, Champagne C, Cooper HL, Vañó-Galván S, Molina-Ruiz AM, Perez NO, Patel GK, Macbeth A, Page M, Bryden A, Mowbray M, Wahie S, Armstrong K, Cooke N, Goodfield M, Man I, de Berker D, Dunnill G, Takwale A, Rao A, Siah TW, Sinclair R, Wade MS, Dlova NC, Setterfield J, Lewis F, Bhargava K, Kirkpatrick N, Estivill X, Stefanato CM, Flohr C, Spector T, Watt FM, Smith CH, Barker JN, Fenton DA, Simpson MA, McGrath JA
Nat Commun 2019 Mar 8;10(1):1150. doi: 10.1038/s41467-019-09117-w. PMID: 30850646Free PMC Article
Resource utilization and quality of life associated with congenital ichthyoses.
Kamalpour L, Gammon B, Chen KH, Veledar E, Pavlis M, Rice ZP, Chen SC
Pediatr Dermatol 2011 Sep-Oct;28(5):512-8. Epub 2011 Sep 5 doi: 10.1111/j.1525-1470.2011.01432.x. PMID: 21895756
Clinical and molecular diagnostic criteria of congenital atrichia with papular lesions.
Zlotogorski A, Panteleyev AA, Aita VM, Christiano AM
J Invest Dermatol 2002 May;118(5):887-90. doi: 10.1046/j.1523-1747.2001.01767.x. PMID: 11982770
Unraveling the molecular mechanisms of hair and nail genodermatoses.
Stratigos AJ, Baden HP
Arch Dermatol 2001 Nov;137(11):1465-71. doi: 10.1001/archderm.137.11.1465. PMID: 11708949
Ichthyosis follicularis, alopecia, and photophobia (IFAP) syndrome: clinical and neuropathological observations in a 33-year-old man.
Keyvani K, Paulus W, Traupe H, Kiesewetter F, Cursiefen C, Huk W, Raab K, Orth U, Rauch A, Pfeiffer RA
Am J Med Genet 1998 Jul 24;78(4):371-7. doi: 10.1002/(sici)1096-8628(19980724)78:4<371::aid-ajmg13>3.0.co;2-f. PMID: 9714442

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