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Becker nevus syndrome(BNS)

MedGen UID:
347608
Concept ID:
C1858042
Disease or Syndrome
Synonyms: BNS; Unilateral hypoplasia of breast musculoskeletal defects
 
Gene (location): ACTB (7p22.1)
 
Monarch Initiative: MONDO:0011500
OMIM®: 604919
Orphanet: ORPHA64755

Definition

Becker nevus (BN) is a cutaneous hamartoma affecting approximately 1 in 200 individuals that appears in childhood as a unilateral tan patch, and increases in thickness, pigmentation, and hair growth during adolescence. Histologically, epidermal acanthosis is accompanied by irregularly dispersed ectopic smooth muscle bundles and increased terminal hair follicles (summary by Cai et al., 2017). Becker nevus syndrome (BNS) is a phenotype characterized by the presence of a Becker nevus in association with unilateral hypoplasia of breast or other cutaneous, muscular, or skeletal defects (Happle and Koopman, 1997). [from OMIM]

Clinical features

From HPO
Scoliosis
MedGen UID:
11348
Concept ID:
C0036439
Disease or Syndrome
The presence of an abnormal lateral curvature of the spine.
Cervical rib
MedGen UID:
102359
Concept ID:
C0158779
Congenital Abnormality
Presence of rib formation in the cervical region.
Hemivertebrae
MedGen UID:
82720
Concept ID:
C0265677
Congenital Abnormality
Absence of one half of the vertebral body.
Pectus excavatum
MedGen UID:
781174
Concept ID:
C2051831
Finding
A defect of the chest wall characterized by a depression of the sternum, giving the chest ("pectus") a caved-in ("excavatum") appearance.
Nevus
MedGen UID:
45074
Concept ID:
C0027960
Neoplastic Process
A nevus is a type of hamartoma that is a circumscribed stable malformation of the skin.
Unilateral breast hypoplasia
MedGen UID:
335016
Concept ID:
C1844722
Finding
Underdevelopment of the breast on one side only.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVBecker nevus syndrome
Follow this link to review classifications for Becker nevus syndrome in Orphanet.

Professional guidelines

PubMed

Wu PR, Liu LJ, Zhang YX, Liu Y, Lin XX, Ma G
J Dermatolog Treat 2021 May;32(3):334-339. Epub 2019 Sep 2 doi: 10.1080/09546634.2019.1656795. PMID: 31416363
Patel P, Malik K, Khachemoune A
Am J Clin Dermatol 2015 Jun;16(3):197-204. doi: 10.1007/s40257-015-0123-y. PMID: 25782676

Recent clinical studies

Therapy

Wu PR, Liu LJ, Zhang YX, Liu Y, Lin XX, Ma G
J Dermatolog Treat 2021 May;32(3):334-339. Epub 2019 Sep 2 doi: 10.1080/09546634.2019.1656795. PMID: 31416363
Zhong Y, Chen Y, Huang L, Wang H, Yan T, Yang B, Man MQ
J Am Acad Dermatol 2019 Jan;80(1):e3. Epub 2018 Jul 24 doi: 10.1016/j.jaad.2018.07.016. PMID: 30048662
Momen S, Mallipeddi R, Al-Niaimi F
J Cosmet Laser Ther 2016 Aug;18(4):188-92. Epub 2016 Mar 11 doi: 10.3109/14764172.2015.1114647. PMID: 26735085
Juhl M, Pappo E, Bain M
Dermatol Online J 2015 Aug 15;21(8) PMID: 26437169
Lapidoth M, Adatto M, Cohen S, Ben-Amitai D, Halachmi S
Lasers Med Sci 2014 Jan;29(1):191-3. Epub 2013 Apr 5 doi: 10.1007/s10103-013-1314-5. PMID: 23558435

Prognosis

Patrizi A, Medri M, Raone B, Bianchi F, Aprile S, Neri I
Pediatr Dermatol 2012 Sep-Oct;29(5):571-4. Epub 2012 Apr 4 doi: 10.1111/j.1525-1470.2012.01734.x. PMID: 22471889

Clinical prediction guides

Wu PR, Liu LJ, Zhang YX, Liu Y, Lin XX, Ma G
J Dermatolog Treat 2021 May;32(3):334-339. Epub 2019 Sep 2 doi: 10.1080/09546634.2019.1656795. PMID: 31416363
Sheng P, Cheng YL, Cai CC, Wu YY, Shi G, Zhou Y, Fan YM
Acta Derm Venereol 2018 Oct 10;98(9):867-872. doi: 10.2340/00015555-2986. PMID: 29972220
Sciallis GF, Sciallis AP
Arch Dermatol 2010 Dec;146(12):1408-12. doi: 10.1001/archdermatol.2010.369. PMID: 21173323

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