Becker's Nevus Syndrome

Maria Elena Cucuzza; Sara Paternò; Daniele Attardo; Andrea D. Praticò; Stefano Catanzaro; Agata Polizzi; Carmelo Schepis; Francesco Lacarrubba; Giuseppe Micali; Anna Elisa Verzì; Concetta Pirrone; Elena Commodari; Antonio Zanghì; Stefania Salafia; Elena R. Praticò; Ignacio Pascual-Castroviejo; Martino Ruggieri

doi:10.1055/s-0038-1667168

Journal of Pediatric Neurology, Table of Contents

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^*

¹Unit 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^*

¹Unit 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

¹Unit 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

¹Unit 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

²Maurice Woh Clinical Neuroscience Institute, King's College London, London, United Kingdom

,

Stefano Catanzaro

¹Unit 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

³Instiute of Neurological Science, National Research Council, Catania, Italy

,

Carmelo Schepis

⁴Unit of Dermatology, Oasi Research Institute - IRCCS, Troina, Enna, Italy

,

Francesco Lacarrubba

⁵Dermatology Clinic, University of Catania, Catania, Italy

,

Giuseppe Micali

⁵Dermatology Clinic, University of Catania, Catania, Italy

,

Anna Elisa Verzì

⁵Dermatology Clinic, University of Catania, Catania, Italy

,

Concetta Pirrone

⁶Section of Psychology, Department of Educational Sciences University of Catania, Catania, Italy

,

Elena Commodari

⁶Section of Psychology, Department of Educational Sciences University of Catania, Catania, Italy

,

Antonio Zanghì

⁷Department of Medical and Surgical Sciences and Advanced Technology “G.F. Ingrassia,” University of Catania, Catania, Italy

,

Stefania Salafia

⁸Unit of Pediatrics, Lentini Hospital, Lentini, Italy

,

Elena R. Praticò

⁹Unit of Pediatrics, Carpi Hospital, Carpi, Italy

,

Ignacio Pascual-Castroviejo

¹⁰Servicio de Neurología, Hospital Universitario La Paz, Madrid, España

,

Martino Ruggieri

¹Unit 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

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

Full Text

References

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