Becker nevus syndrome.

Hospital Central da Polícia Militar do Rio de Janeiro, RJ, Brasil.
Anais brasileiros de dermatologia (Impact Factor: 0.72). 06/2010; 85(3):379-84. DOI: 10.1016/S0926-9959(98)95669-0
Source: PubMed


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.

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    ABSTRACT: Becker nevus syndrome is a phenotype characterised by the simultaneous occurrence of: (1) a circumscribed patch of (light or dark brown) hyperpigmentation with a sharply outlined but irregular border (resolving into small spots reminiscent of an archipelago) and hypertrichosis (with increased smooth muscle bundles) with slight acanthosis (the so-called Becker’s nevus); (2) associated unilateral hypoplasia of one or more of the following: breast, underlying musculature (mostly the shoulder girdle), underlying adipose tissue (lipoatrophy) and limb (usually the arm); and (3) underlying skeletal anomalies including vertebral defects and scoliosis, fused or accessory cervical ribs, pectus excavatum or carenatum, and internal tibial torsion (Danarti et al. 2004, Happle et al. 1997, Sugarman 2004). All of these anomalies tend to show a regional correspondence to the nevus and are mostly ipsilateral (Danarti et al. 2004).
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    ABSTRACT: There are several different types of epidermal nevi, including keratinocyte epidermal nevi, nevus sebaceous, nevus comedonicus, and Becker nevus. This article highlights the varied clinical and histologic features of epidermal nevi, discusses recent data on pathogenesis, and provides an update on treatment options. Syndromes associated with epidermal nevi also are described.
    12/2012; 1(4). DOI:10.1007/s13671-012-0025-7
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    ABSTRACT: A clinical entity called "the epidermal nevus syndrome" does not exist. Rather, there are various epidermal nevus syndromes that can be distinguished by clinical, histopathological, and genetic criteria. In this review, five distinct epidermal nevus syndromes, recognizable by different types of associated epithelial nevi, are described. The Schimmelpenning syndrome is characterized by a sebaceous nevus associated with cerebral anomalies, coloboma, and lipodermoid of the conjunctiva. By contrast, cataracts are a prominent feature of the nevus comedonicus syndrome. The pigmented hairy epidermal nevus syndrome includes Becker nevus, ipsilateral hypoplasia of the breast, and skeletal defects such as scoliosis. In the Proteus syndrome, the associated epidermal nevus is of a flat, velvety, nonorganoid type. The CHILD syndrome occurs almost exclusively in girls. The associated CHILD nevus shows unique features such as a diffuse form of lateralization, ptychotropism, and microscopic changes of verruciform xanthoma. The five epidermal nevus syndromes differ in their genetic basis. The Schimmelpenning and nevus comedonicus syndromes are most likely nonhereditary traits. By contrast, the pigmented hairy epidermal nevus syndrome and the Proteus syndrome may be explained by paradominant inheritance. The CHILD syndrome is caused by an X-linked dominant mutation exerting a lethal effect on male embryos. A correct diagnosis of these phenotypes is important for both recognition and treatment of associated anomalies as well as for genetic counseling.
    Seminars in dermatology 07/1995; 14(2):111-21. DOI:10.1016/S1085-5629(05)80006-9
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