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Becker nevus syndrome refers to a rare disorder comprising the typical pigmented lesion and its associated developmental abnormalities. Becker nevus itself is typically localized on the upper trunk, scapular or upper arm unilaterally; however, it can occasionally occur as multiple or bilateral lesions on any parts of the body. In this report, a rare case of multiple Becker nevi arranged in a unique checkerboard-like pattern is presented. More uniquely, the associated abnormalities found in this patient, breast hypoplasia and leg lipodystrophy, seem to be regionally correlated with the nevi. In closing, we would like to raise an easy-to-remember 6B’s mnemonic, which stands for Becker, Breast, Bone, Bowen’s disease, Basal cell carcinoma, and Beta-actin, for the cases of Becker nevus syndrome.

A síndrome do nevo de Becker é considerada um fenótipo caracterizado pela presença fundamental do nevo de Becker associado à hipoplasia mamária unilateral ou a outras alterações cutâneas, esqueléticas e/ou musculares. Geralmente, surge ao nascimento, aumenta significativamente na adolescência e é uma das síndromes que constituem a síndrome do nevo epidérmico. O artigo relata o primeiro caso da literatura brasileira da síndrome do nevo de Becker em uma paciente de 14 anos com associação de nevo de Becker, hipoplasia mamária unilateral e escoliose.

Summary Introduction The association of Becker's nevus with other cutaneous, musculoskeletal or maxillofacial anomalies is called Becker nevus syndrome. Ipsilateral breast hypoplasia is the main reason for female patients to seek medical advice. We present two clinical cases of Becker nevus syndrome with thoracic nevus and ipsilateral breast hypoplasia treated with lipofilling alone (fat grafting). Material and methods For the two consecutive cases of Becker nevus syndrome treated by fat grafts, we present the surgical technique and the outcome at one year follow-up. Fat was harvested with cannula after infiltration. The adipose tissue was prepared with a short centrifugation. Fat grafting was realized as backward injections. Results We have noticed a concomitant improvement of the thoracic nevus color with a stable result after one-year follow-up. The aesthetic result after lipofilling was evaluated as very satisfying by the patient. The breast symmetry was improved. Conclusions We believe that the lipofilling technique is a natural and valuable treatment option for thoracic anomalies in Becker nevus syndrome with a major impact on patient's quality of life.

Resumen El nevo de Becker es un hamartoma hiperpigmentado, de bordes geograficos y a menudo con hipertricosis en superficie. Sus localizaciones habituales suelen ser los hombros o el torax, aunque puede aparecer en cualquier otra ubicacion. En ocasiones el nevo de Becker se asocia a diferentes anomalias musculares, esqueleticas o cutaneas como la hipoplasia ipsilateral de la mama o la escoliosis. A este fenotipo caracteristico de nevo de Becker asociado a hipoplasia unilateral de la mama u otras anomalias se le denomina sindrome del nevo de Becker. Aunque las lesiones suelen manifestarse en la adolescencia, estas estan presentes desde el nacimiento y constituyen una parte del espectro de los llamados sindromes de nevo epidermico. Presentamos 4 casos de sindrome del nevo de Becker, en el que el nevo de Becker se asocio a hipoplasia mamaria ipsilateral y, menos consistentemente, a otras anomalias.

A síndrome do Nevo de Becker consiste na associação do nevo de Becker com hipoplasia mamária e outras alterações ósseas ou musculares ipsilaterais a lesão. È considerada desordem hormônio dependente causada pelo distúrbio na atividade do receptor de androgênios que parece estar aumentado no nevo de Becker podendo influenciar no desenvolvimento das lesões associadas. Apresentamos um caso relevante desta síndrome pela raridade da sistematização das lesões, além do exuberante acometimento extracutâneo neste caso.

Becker nevus syndrome (BNS) is a rare epidermal nevus syndrome characterized with Becker nevus and ipsilateral breast gland hypoplasia or other skin, skeletal and/or muscle tissue disorders. A 24-year-old woman presented with brown, irregular bordered patch with a diameter of approximately 10 cm which consisted of several small macules on the left breast skin. The ultrasonography and magnetic resonance imaging revealed left breast hypoplasia. Histopathological examination demonstrated minimal acanthosis, papillomatosis, increase in basal layer melanin and hypertrophy of the erector pili muscle. Immunohistochemical staining was positive for androgen in the epidermis, dermal stromal cells and skin appendages. Depending on the clinical and histopathological findings, the patient was diagnosed as BNS. Diagnosis of BNS needs careful examination of pigmented macules and patches since non-hairy BN may be easily overlooked. Patients with BN should be evaluated for associated abnormalities of BNS, in which the severity and extend of ectodermal involvement may differ from patient to other.

Background Becker nevus is a nevoid melanosis, referred to as Becker nevus syndrome when it is associated with other anomalies. Our objectives were to report the occurrence of a Becker nevus with an underlying desmoid soft-tissue tumor; to review Mayo Clinic's experience with Becker nevi, concentrating on Becker nevi associated with bone, vascular, neural, and other soft-tissue abnormalities; to inform physicians of the Becker nevus syndrome; and finally to alert clinicians to evaluate a Becker nevus with its associations in mind. Observations A 46-year-old woman had a Becker nevus with an underlying desmoid-type fibromatosis (desmoid tumor) presenting clinically as a “painful dimple” within the nevus. Review of medical records for 1997 through 2006 at Mayo Clinic, Rochester, Minnesota, yielded 52 patients with Becker nevi, 12 of whom had an associated bone, vascular, neural, congenital, or other soft-tissue abnormality, ranging from liposarcoma to an accessory areola. Conclusions We add to the literature a unique case of desmoid-type fibromatosis immediately beneath a Becker melanosis, which presented as a painful dimple. We hope to raise awareness that a Becker nevus may be associated with other abnormalities, including an infiltrative soft-tissue tumor. We also emphasize the importance of follow-up, including inspection of not only the surface but also the deep tissues underlying the Becker nevus.