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Lichen sclerosus et atrophicus(LSA)

MedGen UID:
7338
Concept ID:
C0023652
Disease or Syndrome
Synonyms: Lichen sclerosis; Lichen sclerosis et atrophicus; Lichen sclerosus; LSA
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
892334
Concept ID:
CN000007
Functional Concept
Source: HPO
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
SNOMED CT: Lichen sclerosus (25674000); Lichen sclerosus et atrophicus (25674000); White spot disease (25674000)
 
OMIM®: 151590

Definition

A chronic inflammatory mucocutaneous disease usually affecting the female genitalia (VULVAR LICHEN SCLEROSUS) and BALANITIS XEROTICA OBLITERANS in males. It is also called white spot disease and Csillag's disease. [from MeSH]

Clinical features

Squamous cell carcinoma
MedGen UID:
505376
Concept ID:
CN002585
Finding
The presence of squamous cell carcinoma of the skin.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLichen sclerosus et atrophicus

Recent clinical studies

Etiology

Erickson BA, Elliott SP, Myers JB, Voelzke BB, Smith TG 3rd, McClung CD, Alsikafi NF, Vanni AJ, Brant WO, Broghammer JA, Tam CA, Zhao LC, Buckley JC, Breyer BN; Trauma and Urologic Reconstructive Network of Surgeons
J Urol 2016 Feb;195(2):363-8. Epub 2015 Sep 5 doi: 10.1016/j.juro.2015.08.096. [Epub ahead of print] PMID: 26343349Free PMC Article
Tong LX, Sun GS, Teng JM
Pediatr Dermatol 2015 Sep-Oct;32(5):593-9. Epub 2015 May 4 doi: 10.1111/pde.12615. [Epub ahead of print] PMID: 25940739
Homer L, Buchanan KJ, Nasr B, Losty PD, Corbett HJ
J Urol 2014 Dec;192(6):1784-8. Epub 2014 Jun 30 doi: 10.1016/j.juro.2014.06.077. [Epub ahead of print] PMID: 24992332
Liu JS, Walker K, Stein D, Prabhu S, Hofer MD, Han J, Yang XJ, Gonzalez CM
J Urol 2014 Sep;192(3):775-9. Epub 2014 Mar 21 doi: 10.1016/j.juro.2014.03.090. [Epub ahead of print] PMID: 24657836
Alonso-Llamazares J, Persing DH, Anda P, Gibson LE, Rutledge BJ, Iglesias L
Acta Derm Venereol 1997 Jul;77(4):299-304. PMID: 9228224

Diagnosis

Liu Y, Hua H, Gao Y
Chin J Dent Res 2013;16(2):157-60. PMID: 24436952
Marcus-Braun N, Hasan Z, Szvalb S, Ben Shachar I
J Pediatr Adolesc Gynecol 2013 Dec;26(6):e117-9. Epub 2013 May 29 doi: 10.1016/j.jpag.2013.03.006. [Epub ahead of print] PMID: 23726137
Viana Fde O, Cavaleiro LH, Unger DA, Miranda MF, Brito AC
An Bras Dermatol 2011 Jul-Aug;86(4 Suppl 1):S82-4. PMID: 22068779
Stewart L, Nuara A, Anand D, Kia K, Cockerell CJ
Arch Dermatol 2011 Jul;147(7):857-62. doi: 10.1001/archdermatol.2011.165-a. PMID: 21768488
Carneiro S, Ramos-e-Silva M, Russi DC, Albuquerque EM, Sousa MA
Skinmed 2011 Mar-Apr;9(2):131-3. PMID: 21548525

Therapy

Tong LX, Sun GS, Teng JM
Pediatr Dermatol 2015 Sep-Oct;32(5):593-9. Epub 2015 May 4 doi: 10.1111/pde.12615. [Epub ahead of print] PMID: 25940739
Homer L, Buchanan KJ, Nasr B, Losty PD, Corbett HJ
J Urol 2014 Dec;192(6):1784-8. Epub 2014 Jun 30 doi: 10.1016/j.juro.2014.06.077. [Epub ahead of print] PMID: 24992332
Viana Fde O, Cavaleiro LH, Unger DA, Miranda MF, Brito AC
An Bras Dermatol 2011 Jul-Aug;86(4 Suppl 1):S82-4. PMID: 22068779
Stewart L, Nuara A, Anand D, Kia K, Cockerell CJ
Arch Dermatol 2011 Jul;147(7):857-62. doi: 10.1001/archdermatol.2011.165-a. PMID: 21768488
Mann DJ, Vergilis-Kalner IJ, Wasserman JR, Petronic-Rosic V
Skinmed 2010 Jul-Aug;8(4):242-4. PMID: 21137615

Prognosis

Surkan M, Hull P
J Cutan Med Surg 2015 Nov-Dec;19(6):600-3. Epub 2015 Jun 8 doi: 10.1177/1203475415590735. [Epub ahead of print] PMID: 26054578
Arps DP, Chen S, Fullen DR, Hristov AC
Arch Pathol Lab Med 2014 Oct;138(10):1319-27. doi: 10.5858/arpa.2014-0298-CC. PMID: 25268195
Homer L, Buchanan KJ, Nasr B, Losty PD, Corbett HJ
J Urol 2014 Dec;192(6):1784-8. Epub 2014 Jun 30 doi: 10.1016/j.juro.2014.06.077. [Epub ahead of print] PMID: 24992332
Liu JS, Walker K, Stein D, Prabhu S, Hofer MD, Han J, Yang XJ, Gonzalez CM
J Urol 2014 Sep;192(3):775-9. Epub 2014 Mar 21 doi: 10.1016/j.juro.2014.03.090. [Epub ahead of print] PMID: 24657836
Osiecka BJ, Nockowski P, Jurczyszyn K, Ziólkowski P
Photodiagnosis Photodyn Ther 2012 Jun;9(2):186-8. Epub 2012 Mar 27 doi: 10.1016/j.pdpdt.2012.02.002. [Epub ahead of print] PMID: 22594990

Clinical prediction guides

Erickson BA, Elliott SP, Myers JB, Voelzke BB, Smith TG 3rd, McClung CD, Alsikafi NF, Vanni AJ, Brant WO, Broghammer JA, Tam CA, Zhao LC, Buckley JC, Breyer BN; Trauma and Urologic Reconstructive Network of Surgeons
J Urol 2016 Feb;195(2):363-8. Epub 2015 Sep 5 doi: 10.1016/j.juro.2015.08.096. [Epub ahead of print] PMID: 26343349Free PMC Article
Surkan M, Hull P
J Cutan Med Surg 2015 Nov-Dec;19(6):600-3. Epub 2015 Jun 8 doi: 10.1177/1203475415590735. [Epub ahead of print] PMID: 26054578
Skupsky H, Abuav R, High W, Pass C, Goldenberg G
J Cutan Pathol 2010 Aug;37(8):877-80. Epub 2009 Aug 23 doi: 10.1111/j.1600-0560.2009.01398.x. [Epub ahead of print] PMID: 19703239
Lukowsky A, Muche JM, Sterry W, Audring H
J Invest Dermatol 2000 Aug;115(2):254-9. doi: 10.1046/j.1523-1747.2000.00040.x. PMID: 10951243
Cruces MJ, De La Torre C, Losada A, Ocampo C, García-Doval I
Arch Dermatol 1998 Sep;134(9):1118-20. PMID: 9762025

Recent systematic reviews

Kirtschig G, Becker K, Günthert A, Jasaitiene D, Cooper S, Chi CC, Kreuter A, Rall KK, Aberer W, Riechardt S, Casabona F, Powell J, Brackenbury F, Erdmann R, Lazzeri M, Barbagli G, Wojnarowska F
J Eur Acad Dermatol Venereol 2015 Oct;29(10):e1-43. Epub 2015 Jul 22 doi: 10.1111/jdv.13136. [Epub ahead of print] PMID: 26202852
Bercaw-Pratt JL, Boardman LA, Simms-Cendan JS; North American Society for Pediatric and Adolescent Gynecology
J Pediatr Adolesc Gynecol 2014 Apr;27(2):111-6. doi: 10.1016/j.jpag.2013.11.004. PMID: 24602304
Xu YM, Feng C, Sa YL, Fu Q, Zhang J, Xie H
Urology 2014 Jan;83(1):232-6. Epub 2013 Nov 5 doi: 10.1016/j.urology.2013.08.035. [Epub ahead of print] PMID: 24200196
Chi CC, Kirtschig G, Baldo M, Lewis F, Wang SH, Wojnarowska F
J Am Acad Dermatol 2012 Aug;67(2):305-12. Epub 2012 Apr 6 doi: 10.1016/j.jaad.2012.02.044. [Epub ahead of print] PMID: 22483994
Garaffa G, Shabbir M, Christopher N, Minhas S, Ralph DJ
J Sex Med 2011 Apr;8(4):1246-53. Epub 2011 Jan 6 doi: 10.1111/j.1743-6109.2010.02165.x. [Epub ahead of print] PMID: 21210959

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