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: 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.
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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.
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    ABSTRACT: We report a case of giant Becker's naevus, ipsilateral areolar hypoplasia and limb asymmetry in a 48-year-old male. This is the first case in which Becker's naevus with areolar hypoplasia has been reported to be associated with other defects. Becker's naevus is a unilateral, hairy, pigmented cutaneous hamartoma which normally appears in adolescence. It was first described by Becker in 19491 and is relatively common affecting 0·5% of males.2 Associated abnormalities have occasionally been reported, such as ipsilateral bread hypoplasia in females,3–7 ipsilateral arm shortening,3,7–9 ipsilateral foot hyperplasia,10 scoliosis and vertebral defects,4,11,12 pectus earinaium,3 pectus excavatum9 and spina bifida,3,13 There has been a recent report of ipsilateral arcolar hypoplasia in three males with Becker's naevus.14
    No preview · Article · Sep 1997 · Clinical and Experimental Dermatology
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