Becker nevus syndrome

A rare, syndromic, benign, epidemal nevus syndrome characterized by the association of a Becker nevus (i.e. circumscribed, unilateral, irregularly shaped, hyperpigmented macules, with or without hypertrichosis and/or acneiform lesions, occuring predominantly on the anterior upper trunk or scapular region) with ipsilateral breast hypoplasia or other, typically hypoplastic, skeletal, cutaneous, and/or muscular defects, such as pectoralis major hypoplasia, supernumerary nipples, vertebral defects, scoliosis, limb asymmetry, odontomaxillary hypoplasia and lipoatrophy.



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Total: 7 (papers)

   


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Matched Phenotype  Gene  Mutation  MeSH
Rank
(Similarity)		 PMID
(PMCID)
1
(23.3%)		 20676475
 Becker nevus syndrome.
Cosendey FE, Martinez NS, Bernhard GA, Dias MF, Azulay DR.
An Bras Dermatol. 2010;85(3):379-84.
Scoliosis
Breast Females Hamartoma Homo sapiens Hyperpigmentation Hypertrichosis Syndrome
1
(23.3%)		 17961453
 [Becker nevus syndrome].
Alfaro A, Torrelo A, Hernandez A, Zambrano A, Happle R.
Actas Dermosifiliogr. 2007;98(9):624-6.
Scoliosis
Breast Child Hamartoma Homo sapiens Hyperpigmentation Male Nevus Syndrome
3
(17.5%)		 31218086
 A Rare Case of Checkerboard-Like Becker Nevi with a Unique Distribution of Abnormalities.
Wongtada C, Asawanonda P, Pongprutthipan M.
Case Rep Dermatol Med. 2019;2019:2387365.
Basal cell carcinoma Lipodystrophy
4
(4.0%)		 25746302
 Breast lipofilling: A new treatment of Becker nevus syndrome.
Ho Quoc C, Fakiha M, Meruta A, Dlimi C, Piat JM, Delay E.
Ann Chir Plast Esthet. 2015;60(4):336-9.
Breast hypoplasia
Adult Breast Females Homo sapiens Nevus Skin Neoplasms Young Adult
4
(4.0%)		 25162246
 [The interesting case: unilateral hypoplasia of the breast in Becker nevus syndrome].
Schneider ME, Draths R, Fritsche E, Hug U.
Handchir Mikrochir Plast Chir. 2014;46(4):266-7.
Breast hypoplasia
Breast Females Homo sapiens Nevus Skin Neoplasms Young Adult
4
(4.0%)		 21437545
 Do you know this syndrome? Becker nevus syndrome.
Steiner D, Silva FA, Pessanha AC, Bialeski N, Feola C, Buzzoni CA.
An Bras Dermatol. 2011;86(1):165-6.
Breast hypoplasia
AR
Biopsy Hamartoma Homo sapiens Male Melanosis Skin Syndrome
4
(4.0%)		 21173323
 Becker nevus with an underlying desmoid tumor: a case report and review including Mayo Clinic's experience.
Sciallis GF, Sciallis AP.
Arch Dermatol. 2010;146(12):1408-12.
Nevus
Back Differential Diagnosis Females Fibromatosis, Aggressive Homo sapiens Magnetic Resonance Imaging Middle Aged Neoplasms, Multiple Primary Skin Neoplasms Soft Tissue Neoplasms Syndrome
        

Phenotype(s) retrieved from Orphanet

    Total: 19

HPO ID Term Frequency
HP:0001034 Hypermelanotic macule Very frequent (99-80%)
HP:0002558 Supernumerary nipple Very frequent (99-80%)
HP:0002983 Micromelia Very frequent (99-80%)
HP:0003724 Shoulder girdle muscle atrophy Very frequent (99-80%)
HP:0010566 Hamartoma Very frequent (99-80%)
HP:0100578 Lipoatrophy Very frequent (99-80%)
HP:0000767 Pectus excavatum Frequent (79-30%)
HP:0000768 Pectus carinatum Frequent (79-30%)
HP:0010311 Aplasia/Hypoplasia of the breasts Frequent (79-30%)
HP:0000045 Abnormality of the scrotum Occasional (29-5%)
HP:0000064 Hypoplastic labia minora Occasional (29-5%)
HP:0000902 Rib fusion Occasional (29-5%)
HP:0002650 Scoliosis Occasional (29-5%)
HP:0002808 Kyphosis Occasional (29-5%)
HP:0002992 Abnormality of tibia morphology Occasional (29-5%)
HP:0003298 Spina bifida occulta Occasional (29-5%)
HP:0005815 Supernumerary ribs Occasional (29-5%)
HP:0100559 Lower limb asymmetry Occasional (29-5%)
HP:0100560 Upper limb asymmetry Occasional (29-5%)


Phenotype(s) retrieved from case reports

    Total: 5

HPO ID Term # of case reports
HP:0003187 Breast hypoplasia 5
HP:0003764 Nevus 5
HP:0002650 Scoliosis 1
HP:0002671 Basal cell carcinoma 1
HP:0012813 Unilateral breast hypoplasia 1


Causative gene(s) retrieved from Orphanet

    Total: 1

Gene Symbol Gene Name Entrez Gene ID
ACTB actin beta 60