Becker’s Nevus Syndrome in a Pediatric Female Patient

Becker’s nevus syndrome is part of the epidermal nevus syndromes and has been described with a phenotype that includes Becker’s nevus, ipsilateral breast hypoplasia, and variable skeletal malformations. It is more frequent in males than in females (5 : 1) but is more relevant in females. The diagnosis is clinically based and the skin lesion must be present and no other numbered criteria have been established, but with more criteria being present the possibility of the diagnosis is higher. Regarding the treatment of breast hypoplasia, the use of antiandrogen medication has demonstrated adequate clinical response in a dose of 50 mg/day of spironolactone.

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Becker’s nevus syndrome: a case report

Journal of Medical Case Reports ◽

10.1186/s13256-021-02996-y ◽

2021 ◽

Vol 15 (1) ◽

Author(s):

Ugo N. Chikani ◽

Ijeoma N. Ohuche ◽

Ada I. Bisi-Onyemaechi

Keyword(s):

Case Report ◽

Female Patient ◽

Pediatric Endocrinology ◽

Ipsilateral Breast ◽

Epidermal Nevus ◽

Case Presentation ◽

Breast Hypoplasia ◽

Becker’S Nevus ◽

Nigeria Teaching Hospital ◽

Endocrinology Clinic

Abstract Background Becker’s nevus syndrome is a syndrome characterized by the presence of a Becker’s nevus with ipsilateral breast hypoplasia or hypoplastic defects of the muscle, skin, or skeleton. The nevus usually consists of a circumscribed, unilateral, irregularly shaped hyperpigmented macule, commonly occurring around the anterior upper trunk, with/without hypertrichosis and/or acneiform lesions. This rare syndrome has not been reported in our locality to the best of our knowledge. Case presentation We report the case of a 15-year-old Igbo female patient who presented to our pediatric endocrinology clinic, University of Nigeria Teaching Hospital, Enugu, with complaints of asymmetry of the breasts and hyperpigmented macules on the side. Based on her symptoms, diagnosis of Becker’s nevus syndrome was made. The diagnosis of Becker’s nevus syndrome is mostly clinical, based on the presence of a Becker’s nevus with ipsilateral breast hypoplasia or hypoplastic defects of the muscle, skin, or skeleton. In our patient, there was a Becker’s nevus with ipsilateral breast hypoplasia. This syndrome, belonging to the class of epidermal nevus syndromes, is very rare, and is usually benign. She was placed on spironolactone tablets 50 mg daily, which have been associated with an improvement in the size of the hypoplastic breast, and her fears were allayed. Conclusion This syndrome has not been reported in our locality to the best of our knowledge and, therefore, has a propensity for misdiagnosis by clinicians because of its rarity. We therefore report this to create awareness among clinicians regarding this condition that is associated with much psychosocial trauma among patients, and that can be easily managed with oral spironolactone.

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Becker's Nevus and Ipsilateral Breast Hypoplasia Androgen-Receptor Study in Two Patients

Archives of Dermatology ◽

10.1001/archderm.1992.01680170128025 ◽

1992 ◽

Vol 128 (7) ◽

pp. 992 ◽

Cited By ~ 15

Author(s):

M. Formigón

Keyword(s):

Androgen Receptor ◽

Ipsilateral Breast ◽

Breast Hypoplasia ◽

Becker’S Nevus

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Becker's nevus and ipsilateral breast hypoplasia--androgen-receptor study in two patients

Archives of Dermatology ◽

10.1001/archderm.128.7.992 ◽

1992 ◽

Vol 128 (7) ◽

pp. 992-993 ◽

Cited By ~ 9

Author(s):

M. Formigon

Keyword(s):

Androgen Receptor ◽

Ipsilateral Breast ◽

Breast Hypoplasia ◽

Becker’S Nevus

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Becker's nevus with ipsilateral breast hypoplasia

Indian Journal of Dermatology Venereology and Leprology ◽

10.4103/0378-6323.48683 ◽

2009 ◽

Vol 75 (2) ◽

pp. 202 ◽

Cited By ~ 2

Author(s):

CS Sirka ◽

MR Puhan ◽

S Behera ◽

P Mohanty ◽

M Nanda

Keyword(s):

Ipsilateral Breast ◽

Breast Hypoplasia ◽

Becker’S Nevus

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Becker's Nevus with Ipsilateral Breast Hypoplasia: Improvement with Spironolactone

The Journal of Dermatology ◽

10.1111/j.1346-8138.2003.tb00365.x ◽

2003 ◽

Vol 30 (2) ◽

pp. 154-156 ◽

Cited By ~ 19

Author(s):

Jae Hoon Jung ◽

You Chan Kim ◽

Hyang Joon Park ◽

Yong Woo Cinn

Keyword(s):

Ipsilateral Breast ◽

Breast Hypoplasia ◽

Becker’S Nevus

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Keratinocytic epidermal nevus with ipsilateral breast hypoplasia

International Journal of Women s Dermatology ◽

10.1016/j.ijwd.2018.12.003 ◽

2019 ◽

Vol 5 (3) ◽

pp. 181-182

Author(s):

Eric Chen ◽

Anthony J. Chiaravalloti ◽

Justin Finch

Keyword(s):

Ipsilateral Breast ◽

Epidermal Nevus ◽

Breast Hypoplasia

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Epidermal nevus with ipsilateral breast hypoplasia

Journal of the American Academy of Dermatology ◽

10.1016/j.jaad.2017.04.484 ◽

2017 ◽

Vol 76 (6) ◽

pp. AB124

Keyword(s):

Ipsilateral Breast ◽

Epidermal Nevus ◽

Breast Hypoplasia

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Becker's Nevus Syndrome

Journal of Pediatric Neurology ◽

10.1055/s-0038-1667168 ◽

2018 ◽

Vol 16 (05) ◽

pp. 352-361 ◽

Cited By ~ 2

Author(s):

Maria Cucuzza ◽

Sara Paternò ◽

Stefano Catanzaro ◽

Agata Polizzi ◽

Carmelo Schepis ◽

...

Keyword(s):

Natural History ◽

Soft Tissues ◽

Cutaneous Lesion ◽

Simultaneous Occurrence ◽

Pectus Carinatum ◽

Epidermal Nevus ◽

Becker’S Nevus ◽

History Of ◽

Prepubertal Boys ◽

Postzygotic Mutations

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.

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Dermoscopy Case of the Month

Serbian Journal of Dermatology and Venerology ◽

10.1515/sjdv-2017-0015 ◽

2017 ◽

Vol 9 (3) ◽

pp. 135-139

Author(s):

Tatjana Roš ◽

Marijana Badrkić ◽

Marijana Macanović ◽

Nenad Šolajić ◽

Branislava Gajić

Keyword(s):

Breast Carcinoma ◽

Female Patient ◽

Paget’S Disease ◽

Paget's Disease ◽

Ipsilateral Breast ◽

Classical Form ◽

Nipple Areola Complex ◽

Areola Complex ◽

Intraepithelial Carcinoma ◽

Mammary Paget’S Disease

AbstractMammary Paget's disease is a rare intraepithelial carcinoma of the nipple/areola complex often associated with ductal breast carcinoma. We report a case of a 55-year-old female patient with a classical form of mammary Paget's disease associated ductal ipsilateral breast carcinoma. Dermoscopy of Paget's disease revealed a whitish-pink area with polymorphous vessels organized in irregular nests separated by pale streak-like structures, with peripheral light brown diffuse pigmentation. Dermoscopic features described in this case are in agreement with rare previous reports and may contribute to better differentiation of mammary Paget's disease from clinically similar lesions.

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