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class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 2 => array:3 [ "nombre" => "Claúdia Fernandes" "apellidos" => "Lorea" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "aff0015" ] ] ] 3 => array:3 [ "nombre" => "Valéria Magalhães" "apellidos" => "Jorge" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">d</span>" "identificador" => "aff0020" ] ] ] 4 => array:3 [ "nombre" => "Antônia Larangeira" "apellidos" => "de Almeida" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">e</span>" "identificador" => "aff0025" ] ] ] ] "afiliaciones" => array:5 [ 0 => array:3 [ "entidad" => "Post-Graduation Program in Health Sciences, Universidade Católica de Pelotas, Pelotas, RS, Brazil" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Department of Dermatology, Universidade Federal de Pelotas and Universidade Católica de Pelotas, Pelotas, RS, Brazil" "etiqueta" => "b" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Genetics, Universidade Federal de Pelotas, Pelotas, RS, Brazil" "etiqueta" => "c" "identificador" => "aff0015" ] 3 => array:3 [ "entidad" => "Adjunct Professor of Pathology, Universidade Federal de Pelotas, Pelotas, RS, Brazil" "etiqueta" => "d" "identificador" => "aff0020" ] 4 => array:3 [ "entidad" => "Dermatology League, Universidade Federal de Pelotas, Pelotas, RS, Brazil" "etiqueta" => "e" "identificador" => "aff0025" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:8 [ "identificador" => "fig0015" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 1641 "Ancho" => 3340 "Tamanyo" => 1194144 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0015" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Transmission electron microscopy (A), basal keratinocyte with hemidesmosomes and normal basement membrane (arrows) (×30,000). (B), Large amount of heterogen melanosomes with normal size and normal desmossomes (arrows) (×30,000).</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">McCune<span class="elsevierStyleItalic">-Albright Syndrome (MAS) – OMIM</span> #174800 – is a genetic disease with cutaneous mosaicism caused by post-zygotic activating mutations in GNAS locus, encoding G<span class="elsevierStyleInf">s</span>α protein.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,2</span></a> MAS was originally described in 1936 as a triad of fibrous dysplasia of bone, <span class="elsevierStyleItalic">café-au-lait</span> skin macules, and precocious puberty. However, it is now recognized that its phenotype is far more complex.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> Other endocrinopathies, including hyperthyroidism, estrogen-producing ovarian cysts, growth hormone excess, renal phosphate wasting with or without rickets/osteomalacia, and Cushing syndrome could be seen in association with the original triad.<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2,4</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">MAS is most often identified by dermatologic findings. <span class="elsevierStyleItalic">Café-au-lait</span> macules appear in the neonatal period or in the first months of life. These macules have irregular borders (described as “coast of Maine”) and are unilateral.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">There are yet substantial knowledge gaps about MAS pathophysiology and natural history<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> and very little information about its <span class="elsevierStyleItalic">café-au-lait</span> macules, which were examined by light microscopy, immunohistochemistry, and Transmission Electron Microscopy (TEM) in this report.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Materials and methods</span><p id="par0020" class="elsevierStylePara elsevierViewall">The authors examined a 22-year-old female patient who presented since birth with segmental hyperchromic <span class="elsevierStyleItalic">café-au-lait</span> spots in the right side of the abdomen, with a chessboard-like distribution, respecting the midline (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>a), extending to the right thigh with geographical contours (<a class="elsevierStyleCrossRef" href="#fig0005">Figs. 1</a>b and c). The patient was submitted to surgical correction of scoliosis in childhood and presented also an ovarian cyst and truncal obesity. She had been diagnosed with neurofibromatosis type 1 in her childhood, however, without the presence of neurofibromas, Lish nodules, or axillary ephelides.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0025" class="elsevierStylePara elsevierViewall">Biopsies were taken from the hyperpigmented area and processed for light microscopy (hematoxylin-eosin and immunohistochemistry with HMB-45 and Melan-A) and for TEM, in the latter, ultrathin sections targeted the epidermis.</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Results</span><p id="par0030" class="elsevierStylePara elsevierViewall">Light microscopy showed increased melanin pigment with HE is staining (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>a), in some areas the pigment was seen also in suprabasal layers (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>b). Immunohistochemistry with melanocytic markers (HMB-45 and Melan-A) revealed a normal number of melanocytes (<a class="elsevierStyleCrossRef" href="#fig0010">Figs. 2</a>c and d).</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0035" class="elsevierStylePara elsevierViewall">TEM demonstrated normal epidermal structures, such as desmosomes, cytokeratin filaments, basement membrane and hemidesmosomes (<a class="elsevierStyleCrossRef" href="#fig0015">Figs. 3</a>a and b). A high amount of melanosomes in keratinocytes was observed (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>b), which measured 0.38 to 0.67 nm, a variation in the form and size could also be observed (<a class="elsevierStyleCrossRef" href="#fig0015">Figs. 3</a>b, <a class="elsevierStyleCrossRef" href="#fig0020">4</a>a and <a class="elsevierStyleCrossRef" href="#fig0025">5</a>).</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0040" class="elsevierStylePara elsevierViewall">With high magnifications indentations in melanosomes’ outline (<a class="elsevierStyleCrossRef" href="#fig0020">Fig. 4</a>a and b) could be seen, as well as an irregular melanosomal contour was observed (<a class="elsevierStyleCrossRef" href="#fig0025">Fig. 5</a>), in contrast to normal melanosomes, which show a regular contour and an oval shape (<a class="elsevierStyleCrossRef" href="#fig0025">Fig. 5</a> inset).</p><elsevierMultimedia ident="fig0020"></elsevierMultimedia><elsevierMultimedia ident="fig0025"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Discussion</span><p id="par0045" class="elsevierStylePara elsevierViewall">The clinical signs of the examined patient are in accordance with the literature, with the segmental <span class="elsevierStyleItalic">café-au-lait</span> macule, bone, and ovarian involvement.</p><p id="par0050" class="elsevierStylePara elsevierViewall">Light microscopy observed an increase in the melanin pigment with a normal number of melanocytes in hyperchromic MAS lesions, showed by melanocyte markers.</p><p id="par0055" class="elsevierStylePara elsevierViewall">There are no reports of ultrastructural studies in <span class="elsevierStyleItalic">café-au-lait</span> macules of MAS, the authors found only one study that evaluated <span class="elsevierStyleItalic">café-au-lait</span> spots using electron microscopy in neurofibromatosis in 1992. No abnormal ultrastructural findings were observed in the melanocytes or epidermal keratinocytes, melanosomes (either in melanocytes or in epidermal keratinocytes) had a normal aspect.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">The authors’ findings have also demonstrated a similar pigment increase. With a high-power view, however, the melanosomes present an abnormal aspect, suggesting a defect in their synthesis in MAS.</p><p id="par0065" class="elsevierStylePara elsevierViewall">This study presents the first description of the electron microscopic features of <span class="elsevierStyleItalic">café-au-lait</span> spots in a patient with MAS, describing ultrastructural changes in melanosomes, which appeared with an irregular outline in high magnifications. Dermatologists should recognize this disorder, given the possibility that these macules seen in MAS can be confused with neurofibromatosis, as in the case described here.</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Financial support</span><p id="par0070" class="elsevierStylePara elsevierViewall">None declared.</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Authors’ contributions</span><p id="par0075" class="elsevierStylePara elsevierViewall">Victor Garcia Neto: Approval of the final version of the manuscript; design and planning of the study; drafting and editing of the manuscript; collection, analysis, and interpretation of data; critical review of the manuscript.</p><p id="par0080" class="elsevierStylePara elsevierViewall">Hiram Larangeira de Almeida Jr.: Approval of the final version of the manuscript; design and planning of the study; drafting and editing of the manuscript; collection, analysis, and interpretation of data; effective participation in research orientation; critical review of the manuscript.</p><p id="par0085" class="elsevierStylePara elsevierViewall">Claúdia Fernandes Lorea: Approval of the final version of the manuscript; design and planning of the study; drafting and editing of the manuscript; collection, analysis, and interpretation of data; critical review of the manuscript.</p><p id="par0090" class="elsevierStylePara elsevierViewall">Valéria Magalhães Jorge: Approval of the final version of the manuscript; design and planning of the study; drafting and editing of the manuscript; collection, analysis, and interpretation of data; critical review of the manuscript.</p><p id="par0095" class="elsevierStylePara elsevierViewall">Antônia Larangeira de Almeida: Approval of the final version of the manuscript; design and planning of the study; drafting and editing of the manuscript; collection, analysis, and interpretation of data; critical review of the manuscript.</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Conflicts of interest</span><p id="par0100" class="elsevierStylePara elsevierViewall">None declared.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:10 [ 0 => array:3 [ "identificador" => "xres1653838" "titulo" => "Abstract" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0005" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec1470070" "titulo" => "Keywords" ] 2 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 3 => array:2 [ "identificador" => "sec0010" "titulo" => "Materials and methods" ] 4 => array:2 [ "identificador" => "sec0015" "titulo" => "Results" ] 5 => array:2 [ "identificador" => "sec0020" "titulo" => "Discussion" ] 6 => array:2 [ "identificador" => "sec0025" "titulo" => "Financial support" ] 7 => array:2 [ "identificador" => "sec0030" "titulo" => "Authors’ contributions" ] 8 => array:2 [ "identificador" => "sec0035" "titulo" => "Conflicts of interest" ] 9 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2021-05-28" "fechaAceptado" => "2021-09-05" "PalabrasClave" => array:1 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec1470070" "palabras" => array:3 [ 0 => "Cafe-au-lait spots" 1 => "Fibrous dysplasia, polyostotic" 2 => "Microscopy, electron, transmission" ] ] ] ] "tieneResumen" => true "resumen" => array:1 [ "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">McCune - Albright syndrome is a genetic disease with cutaneous mosaicism caused by post-zygotic activating mutations in GNAS locus, it has a triad of fibrous bone dysplasia, café-au-lait macules and precocious puberty. We examined a 22-year-old female patient with café au lait spot in right side of the abdomen, with a chessboard - like distribution, extending to right thigh with geographical contours, she has also an ovarian cyst, scoliosis and truncal obesity. Biopsies were taken from the hyperpigmented area and processed for light microscopy and for transmission electron microscopy. Light microscopy showed increased melanin pigment with HE staining. Immunohistochemistry with melanocytic markers (HMB-45 and Melan-A) revealed a normal number of melanocytes. Transmission electron microscopy demonstrated normal epidermal structures, such as desmosomes, cytokeratin filaments and hemidesmosomes. With high magnifications an irregular melanossomal contour was seen, with some indentations in their outline.</p></span>" ] ] "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0010">Study conducted at the Programa de Pós-Graduação em Saúde e Comportamento da Universidade Católica de Pelotas and at the Liga de Dermatologia da Universidade Federal de Pelotas, Pelotas, RS, Brazil.</p>" ] ] "multimedia" => array:5 [ 0 => array:8 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1907 "Ancho" => 2924 "Tamanyo" => 618176 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0005" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">(A), Segmental hyperchromic <span class="elsevierStyleItalic">café-au-lait</span> spot in the abdomen, with a chessboard-like distribution, respecting the midline of the body; (B and C), Segmental hyperchromic <span class="elsevierStyleItalic">café-au-lait</span> in the right thigh with geographical contour.</p>" ] ] 1 => array:8 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 2340 "Ancho" => 3174 "Tamanyo" => 746085 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0010" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Light microscopy. (A), Increased melanin pigment with hematoxylin and eosin staining (×150). (B), Increased melanin pigment with hematoxylin and eosin staining which was also seen in suprabasal layers (×400). (C), Immunohistochemistry with melanocytic marker (Melan A) confirmed an increase of melanin and revealed a normal number of melanocytes (×400). (D), Immunohistochemistry with melanocytic marker (HMB-45) confirmed an increase of melanin and revealed a normal number of melanocytes (×400).</p>" ] ] 2 => array:8 [ "identificador" => "fig0015" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 1641 "Ancho" => 3340 "Tamanyo" => 1194144 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0015" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Transmission electron microscopy (A), basal keratinocyte with hemidesmosomes and normal basement membrane (arrows) (×30,000). (B), Large amount of heterogen melanosomes with normal size and normal desmossomes (arrows) (×30,000).</p>" ] ] 3 => array:8 [ "identificador" => "fig0020" "etiqueta" => "Figure 4" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr4.jpeg" "Alto" => 1228 "Ancho" => 3340 "Tamanyo" => 795567 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0020" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Transmission electron microscopy (A and B). Detail of melanosomes with indentations in their outline (×50,000).</p>" ] ] 4 => array:8 [ "identificador" => "fig0025" "etiqueta" => "Figure 5" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr5.jpeg" "Alto" => 1251 "Ancho" => 1750 "Tamanyo" => 370521 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0025" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Transmission electron microscopy – detail of irregular melanosomes, inset with normal regular oval melanossomes (×50,000).</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:7 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Advances in Models of Fibrous Dysplasia/McCune-Albright Syndrome" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "H. 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