Síndrome do nevo de Becker

Abstract

Abstract:Becker nevus syndrome is a phenotype characterized by the fundamental presence of Becker's nevus with ipsilateral hypoplasia of the breast or other skin, skeletal and/or muscular disorders. This syndrome generally originates at birth, intensifies significantly in adolescence and is one of the syndromes that constitute epidermal nevus syndrome. To the best of our knowledge, this is the first case published in the Brazilian literature of Becker nevus syndrome associated with Becker's nevus, ipsilateral breast hypoplasia and scoliosis in a 14-year-old girl. Keywords: hamartoma; hypertrichosis; nevus; scoliosis. Resumo: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, esqueléti-cas 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.

Becker’s Nevus Syndrome in a Pediatric Female Patient

“…It is included in the group of epidermal nevus syndromes, with Proteus syndrome, nevus comedonicus syndrome, phakomatosis pigmentokeratotica, nevus sebaceous syndrome, and CHILD syndrome [5, 6]. The diagnosis is clinical and must include the presence of Becker's nevus and other skin, muscle, and/or skeletal disorders are also necessary, which may include ipsilateral hypoplasia of shoulder or arm, ipsilateral breast hypoplasia, supernumerary nipple, facial asymmetry, skin hypoplasia of temporal region, spina bifida, spinal fusion, pectus carinatum or excavatum, scoliosis, lipodystrophy, stress fractures, accessory scrotum, contralateral hypoplasia of labia minora, and umbilical hernia [7, 8]. The pathogenesis remains unclear; most cases occur sporadically, but familiar involvement may happen due to incomplete penetrance of autosomal dominant inheritance.…”

Can Tissue Expansion Reconstruction in the Trunk of Children Increase the Risk of Scoliosis?

“…These include cutaneous skeletal hypophosphatemia syndrome, 21 CLOVES syndrome, 22,23 and Becker nevus syndrome. 24 Several case reports also present this unique combination in specific patients. 25,26 However, none of our patients had any history of these syndromes or the constellation of symptoms associated with them.…”

Becker′s nevus syndrome

“…[2] Beckers’s nevus is a cutaneous hamartoma characterized by circumscribed hyperpigmentation with hypertrichosis, which was described by Becker in 1949. [3] Many authors have observed the association of Becker's nevus with other cutaneous, musculoskeletal, or maxillofacial anomalies. However, it was only in 1995 that the term hairy epidermal nevus syndrome or Becker's nevus syndrome was described by Happle as an association of Becker's nevus with unilateral breast hypoplasia and muscle, skin, and/or skeletal abnormalities.…”