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and left arm&#46; The lesion had been present since birth&#46; Physical examination revealed a hyperpigmented macule with well-defined borders&#44; with islands of healthy skin on the periphery and mild hypertrichosis with respect to the rest of the skin&#59; the lesion involved the left cheek&#44; neck&#44; and left arm&#46; The clinical picture was interpreted as BNS&#46; The patient also presented hypoplasia of some teeth homolateral to the nevus&#44; and facial asymmetry and scoliosis&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Case 2</span><p id="par0020" class="elsevierStylePara elsevierViewall">An 18-year-old boy with a past history of severe scoliosis that had been treated surgically visited our department with a hyperpigmented macule on the left scapular region&#59; the lesion had been present since birth &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; The patient reported that the lesion had become more pigmented and hairier over time&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Case 3</span><p id="par0025" class="elsevierStylePara elsevierViewall">A 15-year-old boy visited our department with a hyperpigmented macule that involved the area of the right shoulder and scapula&#59; the lesion had been present since birth&#46; The nevus showed a higher density of closed comedones and moderate hypertrichosis compared with the resto of the skin &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; The patient also presented moderate scoliosis&#44; leading to a diagnosis of BNS&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Case 4</span><p id="par0030" class="elsevierStylePara elsevierViewall">A 44-year-old man visited our department with a large hyperpigmented macule on the left scapular region&#59; the lesion was the result of the coalescing of smaller macules &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46; Clear asymmetry in the trophism of the underlying soft tissue was also observed&#46; A chest x-ray revealed marked scoliosis and the clinical picture was interpreted as BNS&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Comment</span><p id="par0035" class="elsevierStylePara elsevierViewall">BN was first described by SW Becker in 1949&#46; It is an androgen-sensitive hamartoma&#46; It usually presents from birth&#44; although it occasionally only becomes visible over time&#44; typically at puberty&#44; when it may become more pigmented and hairier&#44; due to hormone stimulation&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> This change is more evident in males&#46; In their series of 118 cases&#44; Patrizi et al<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> also found a certain link between evidence of BN and exposure to sunlight&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">BNS is the association of BN with underlying musculoskeletal malformations&#46; It is a rare entity that affects both sexes equally&#44; although this varies in different studies&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> Because BN is more frequently found on the thoracic region&#44; especially the pectoral and scapular regions&#44; it is associated with different abnormalities in this location&#44; including as scoliosis&#44; mammary hypoplasia&#44; and muscular hypotrophy&#46; In general&#44; these disorders are ipsilateral to the nevus&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">6&#8211;10</span></a> The entity should be distinguished from Poland syndrome&#44; which is characterized by hypoplasia or unilateral absence of pectoral muscles&#44; ribs&#44; breast&#44; or nipple&#44; together with homolateral syndactyly&#46; It is occasionally associated with compromised internal organs&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">11&#44;12</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">The clinical spectrum of involvement ranges from subtle manifestations that are difficult to detect clinically&#44; to clear abnormalities&#44; which are generally the reason for a medical consultation&#46; In this regard&#44; women consult more frequently when the BNS involves the breast&#44; due to the resulting asymmetry in the anterior thorax&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">6&#44;10</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">BNS forms part of what are known as epidermal nevus syndromes&#44; together with Schimmelpenning syndrome&#44; nevus comedonicus syndrome&#44; Proteus syndrome&#44; and CHILD syndrome&#46; These syndromes are caused by a hamartomatous proliferation derived from the embryonic ectoderm as the result of anomalous growth of different epidermal structures&#44; depending on the case in question&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">They tend to occur due to sporadic mutations&#46; Heterozygotic individuals are phenotypically normal but may transmit the mutation over several generations&#44; whereas the disease manifests in the presence of 2 somatic mutations during embryogenesis&#44; with a paradominant inheritance pattern&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;6</span></a> Its genetic basis is not fully understood&#44; although it is thought to be caused by a lethal postzygotic mutation of an autosomal gene that survives in mosaic form&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a> Indeed&#44; this entity manifests with characteristics of mosaic phenotype&#44; presenting as a solitary lesion with serrated edges or&#44; less commonly&#44; as a segmented or flag-like lesion&#46; Thus&#44; in members of the same family&#44; it is possible to find individuals with BNS and others with only BN&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a></p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Conflicts of Interest</span><p id="par0060" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest&#46;</p></span></span>"
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Case and Research Letters
Becker Nevus: 4 Cases of a Rare Syndrome
Síndrome del nevo de Becker. Cuatro casos de una rara entidad
L.L. Tirellia,
Autor para correspondencia
ltirelli@hospitalaleman.com

Corresponding author.
, A. Labelb, P.C. Lunaa, M. Larraldec
a Dermatólogas del servicio del Hospital Aleman de Buenos Aires, Argentina
b Residente de dermatología del Hospital Alemán, Argentina
c Jefa del servicio de Dermatología del Hospital Alemán de Buenos Aires, Argentina
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and left arm&#46; The lesion had been present since birth&#46; Physical examination revealed a hyperpigmented macule with well-defined borders&#44; with islands of healthy skin on the periphery and mild hypertrichosis with respect to the rest of the skin&#59; the lesion involved the left cheek&#44; neck&#44; and left arm&#46; The clinical picture was interpreted as BNS&#46; The patient also presented hypoplasia of some teeth homolateral to the nevus&#44; and facial asymmetry and scoliosis&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Case 2</span><p id="par0020" class="elsevierStylePara elsevierViewall">An 18-year-old boy with a past history of severe scoliosis that had been treated surgically visited our department with a hyperpigmented macule on the left scapular region&#59; the lesion had been present since birth &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; The patient reported that the lesion had become more pigmented and hairier over time&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Case 3</span><p id="par0025" class="elsevierStylePara elsevierViewall">A 15-year-old boy visited our department with a hyperpigmented macule that involved the area of the right shoulder and scapula&#59; the lesion had been present since birth&#46; The nevus showed a higher density of closed comedones and moderate hypertrichosis compared with the resto of the skin &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; The patient also presented moderate scoliosis&#44; leading to a diagnosis of BNS&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Case 4</span><p id="par0030" class="elsevierStylePara elsevierViewall">A 44-year-old man visited our department with a large hyperpigmented macule on the left scapular region&#59; the lesion was the result of the coalescing of smaller macules &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46; Clear asymmetry in the trophism of the underlying soft tissue was also observed&#46; A chest x-ray revealed marked scoliosis and the clinical picture was interpreted as BNS&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Comment</span><p id="par0035" class="elsevierStylePara elsevierViewall">BN was first described by SW Becker in 1949&#46; It is an androgen-sensitive hamartoma&#46; It usually presents from birth&#44; although it occasionally only becomes visible over time&#44; typically at puberty&#44; when it may become more pigmented and hairier&#44; due to hormone stimulation&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> This change is more evident in males&#46; In their series of 118 cases&#44; Patrizi et al<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> also found a certain link between evidence of BN and exposure to sunlight&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">BNS is the association of BN with underlying musculoskeletal malformations&#46; It is a rare entity that affects both sexes equally&#44; although this varies in different studies&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> Because BN is more frequently found on the thoracic region&#44; especially the pectoral and scapular regions&#44; it is associated with different abnormalities in this location&#44; including as scoliosis&#44; mammary hypoplasia&#44; and muscular hypotrophy&#46; In general&#44; these disorders are ipsilateral to the nevus&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">6&#8211;10</span></a> The entity should be distinguished from Poland syndrome&#44; which is characterized by hypoplasia or unilateral absence of pectoral muscles&#44; ribs&#44; breast&#44; or nipple&#44; together with homolateral syndactyly&#46; It is occasionally associated with compromised internal organs&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">11&#44;12</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">The clinical spectrum of involvement ranges from subtle manifestations that are difficult to detect clinically&#44; to clear abnormalities&#44; which are generally the reason for a medical consultation&#46; In this regard&#44; women consult more frequently when the BNS involves the breast&#44; due to the resulting asymmetry in the anterior thorax&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">6&#44;10</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">BNS forms part of what are known as epidermal nevus syndromes&#44; together with Schimmelpenning syndrome&#44; nevus comedonicus syndrome&#44; Proteus syndrome&#44; and CHILD syndrome&#46; These syndromes are caused by a hamartomatous proliferation derived from the embryonic ectoderm as the result of anomalous growth of different epidermal structures&#44; depending on the case in question&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">They tend to occur due to sporadic mutations&#46; Heterozygotic individuals are phenotypically normal but may transmit the mutation over several generations&#44; whereas the disease manifests in the presence of 2 somatic mutations during embryogenesis&#44; with a paradominant inheritance pattern&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;6</span></a> Its genetic basis is not fully understood&#44; although it is thought to be caused by a lethal postzygotic mutation of an autosomal gene that survives in mosaic form&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a> Indeed&#44; this entity manifests with characteristics of mosaic phenotype&#44; presenting as a solitary lesion with serrated edges or&#44; less commonly&#44; as a segmented or flag-like lesion&#46; Thus&#44; in members of the same family&#44; it is possible to find individuals with BNS and others with only BN&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a></p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Conflicts of Interest</span><p id="par0060" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest&#46;</p></span></span>"
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        "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as&#58; Tirelli LL&#44; Label A&#44; Luna PC&#44; Larralde M&#46; S&#237;ndrome del nevo de Becker&#46; Cuatro casos de una rara entidad&#46; Actas Dermosifiliogr&#46; 2021&#59;112&#58;282&#8211;283&#46;</p>"
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          "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Hyperpigmented macule with hair over the left shoulder&#46; Note the adjacent surgical scar due to the history of severe scoliosis&#46;</p>"
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          "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Hyperpigmented macule on the right scapular region and shoulder&#44; present since birth&#46; The comedones and hair on the macule appeared at puberty&#46;</p>"
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