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Journal of Pediatric Neurology 2018; 16(05): 352-361
DOI: 10.1055/s-0038-1667168
Review Article
Georg Thieme Verlag KG Stuttgart · New York

Becker's Nevus Syndrome

Maria Elena Cucuzza   *
1   Unit of Rare Diseases of the Nervous System in Childhood, Department of Clinical and Experimental Medicine, Section of Pediatrics and Child Neurospychiatry, University of Catania, Catania, Italy
,
Sara Paternò   *
1   Unit of Rare Diseases of the Nervous System in Childhood, Department of Clinical and Experimental Medicine, Section of Pediatrics and Child Neurospychiatry, University of Catania, Catania, Italy
,
Daniele Attardo
1   Unit of Rare Diseases of the Nervous System in Childhood, Department of Clinical and Experimental Medicine, Section of Pediatrics and Child Neurospychiatry, University of Catania, Catania, Italy
,
Andrea D. Praticò
1   Unit of Rare Diseases of the Nervous System in Childhood, Department of Clinical and Experimental Medicine, Section of Pediatrics and Child Neurospychiatry, University of Catania, Catania, Italy
2   Maurice Woh Clinical Neuroscience Institute, King's College London, London, United Kingdom
,
Stefano Catanzaro
1   Unit of Rare Diseases of the Nervous System in Childhood, Department of Clinical and Experimental Medicine, Section of Pediatrics and Child Neurospychiatry, University of Catania, Catania, Italy
,
Agata Polizzi
3   Instiute of Neurological Science, National Research Council, Catania, Italy
,
Carmelo Schepis
4   Unit of Dermatology, Oasi Research Institute - IRCCS, Troina, Enna, Italy
,
Francesco Lacarrubba
5   Dermatology Clinic, University of Catania, Catania, Italy
,
Giuseppe Micali
5   Dermatology Clinic, University of Catania, Catania, Italy
,
Anna Elisa Verzì
5   Dermatology Clinic, University of Catania, Catania, Italy
,
Concetta Pirrone
6   Section of Psychology, Department of Educational Sciences University of Catania, Catania, Italy
,
Elena Commodari
6   Section of Psychology, Department of Educational Sciences University of Catania, Catania, Italy
,
Antonio Zanghì
7   Department of Medical and Surgical Sciences and Advanced Technology “G.F. Ingrassia,” University of Catania, Catania, Italy
,
Stefania Salafia
8   Unit of Pediatrics, Lentini Hospital, Lentini, Italy
,
Elena R. Praticò
9   Unit of Pediatrics, Carpi Hospital, Carpi, Italy
,
Ignacio Pascual-Castroviejo
10   Servicio de Neurología, Hospital Universitario La Paz, Madrid, España
,
Martino Ruggieri
1   Unit of Rare Diseases of the Nervous System in Childhood, Department of Clinical and Experimental Medicine, Section of Pediatrics and Child Neurospychiatry, University of Catania, Catania, Italy
› Author Affiliations
Further Information

Publication History

05 February 2018

21 May 2018

Publication Date:
20 August 2018 (online)

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Abstract

The simultaneous occurrence of a patch of light or dark brown hyperpigmentation with hypertrichosis (Becker's nevus) together with (usually ipsilateral) soft tissues hypoplasia (especially breast, in women) and underlying skeletal anomalies (i.e., vertebral hypoplasia, scoliosis, pectus carinatum or excavatum) represents the Becker's nevus syndrome (BNS) phenotype. It was first described (as a single cutaneous lesion) by Becker in 1949 and then associated with the surrounding musculoskeletal disorders. The syndrome has also been reported as pigmentary hairy epidermal nevus syndrome. Less than 100 cases have been reported in the literature, with a slightly higher incidence in females and only few familiar cases: paradominant postzygotic mutations and/or an androgen-dependent hyperactivation have been reported as the causes of the diseases.

The extracutaneous lesions are congenital and nonprogressive, and the natural history of the Becker's nevus is the same as that of isolated nevi: in prepubertal boys, the pigmentation may be less intense and the hairiness may be absent or mild, as occurs in women, whereas in men, there is an increase of hairiness after puberty. The treatment is essentially cosmetic, and potential therapeutic options include electrolysis, waxing, makeup, or laser.

Keywords

Becker's nevus - hyperpigmentation - hypertrichosis - soft tissues hypoplasia - skeletal abnormalities

* Both authors contributed equally to the manuscript.