Cases an Research Letters
Pseudoxanthoma Elasticum in the Differential Diagnosis of Axillary Calcifications on Mammography
Seudoxantoma elástico en el diagnóstico diferencial de las calcificaciones axilares en la mamografía
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Padilla-España, T. Fernández-Morano, J. Del Boz, R. Fúnez" "autores" => array:4 [ 0 => array:4 [ "nombre" => "L." "apellidos" => "Padilla-España" "email" => array:1 [ 0 => "eplaura85@hotmail.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">¿</span>" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "T." "apellidos" => "Fernández-Morano" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "J." "apellidos" => "Del Boz" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 3 => array:3 [ "nombre" => "R." "apellidos" => "Fúnez" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Servicio de Dermatología, Hospital Costa del Sol, Marbella, Málaga, Spain" "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Anatomía Patológica, Hospital Costa del Sol, Marbella, Málaga, Spain" "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Seudoxantoma elástico en el diagnóstico diferencial de las calcificaciones axilares en la mamografía" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 530 "Ancho" => 1401 "Tamanyo" => 96182 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">A, Redundant skin folds in the right axilla. B, Redundant skin folds in the left axilla.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">We present the case of a 52-year-old woman with a family history of breast cancer (mother) who came to the hospital for her annual mammography screening. Microcalcifications with a linear distribution following the skin folds were observed, predominantly in the upper outer quadrants of the breasts and in both axillary regions (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">She was referred to the dermatology department for the evaluation of skin changes, which the patient described as having been present for many years. Physical examination revealed conspicuous folds of redundant skin on the neck and in both axillas and groins, with yellow papules on the skin surface giving a “plucked chicken skin” appearance (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>, A and B). Breast palpation revealed small, superficial, stony-hard nodules, particularly in the upper outer quadrants.</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Incisional skin biopsy was performed, attempting to include these nodules. Histology showed fragmentation and calcification of the elastic fibers in the middle and deep thirds of the dermis; these fibers stained blue with hematoxylin-eosin stain due to their high calcium content, and stained a brownish color with von Kossa stain (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>, A and B).</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">A diagnosis of pseudoxanthoma elasticum (PXE) was confirmed on the basis of these findings.</p><p id="par0025" class="elsevierStylePara elsevierViewall">On consultation with the ophthalmology, cardiology, and gastroenterology departments, angioid striae were detected in both eyes, with slight edema in the juxtafoveal area of the macula, and mitral valve prolapse; there were no associated digestive tract changes.</p><p id="par0030" class="elsevierStylePara elsevierViewall">PXE is a hereditary connective tissue disease characterized by variable skin, ocular, and cardiovascular manifestations. The inheritance pattern can be autosomal dominant or recessive, although its incidence is twice as high in women as in men. The locus maps to chromosome 16p13.11.2.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,2</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">The typical skin manifestations consist of the appearance of yellowish macules in the axillas, on the lateral surfaces of the neck, and in the groin. These macules progress to papules that can coalesce to form large plaques and, with the passage of time, the affected skin folds become more lax and more conspicuous. Clinically, the relevant differential diagnosis of these lesions includes xanthoma planum, typically associated with dyslipidemia, actinic elastosis, and the skin lesions observed in patients taking D-penicillamine.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">The most characteristic histological change in the affected organs is the presence of degenerating elastic fibers (fragmentation in the superficial and mid dermis) that become calcified; the absence of such fibers should make us question the diagnosis. The changes in the elastic fibers can be observed after hematoxylin-eosin staining, although von Kossa stain can be useful to better reveal the calcium (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>B) and Verhoeff-Van Gieson stain for the elastic fibers.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">It is therefore not uncommon to observe images with the density of calcium on plain x-rays or mammography in these patients; these images may have a linear distribution when they are produced by calcification of the elastic fibers in blood vessel walls. There may also be microcalcifications in the breasts and axillas due to calcification of the elastic fibers of the dermis,<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> and it has even been suggested that these microcalcifications in the breast could be the result of calcification of the muscle fascia and of the interlobar septa of the gland.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">Microcalcifications on mammography have been reported in numerous diseases, such as Albright osteodystrophy, chronic folliculitis, and osteoma cutis, and in a wide variety of metabolic and endocrine disorders, such as hyperparathyroidism and hypervitaminosis D. The differential diagnosis should also include deposits of metallic substances on the skin due to the use of deodorants and other creams, as these can simulate intracutaneous microcalcifications.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">According to the study by Bercovitch et al.,<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> breast microcalcifications may be detected in more than half of women with PXE. In the majority of cases these calcifications are isolated, although they have been reported to form groups in some patients,<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> creating calcium-containing masses that would explain the presence of palpable nodules in both axillas, as occurred in our patient. The combination of breast microcalcifications and vascular calcifications is highly characteristic in these patients, and although it is not rare to detect either of these in the normal population, their combined presence should make us look in detail at the skin for the possible presence of changes suggestive of PXE. It has been reported that calcifications affecting vascular structures that contain elastic tissue can be observed on plain radiographs in up to a third of patients<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> and calcification of the coronary arteries can give rise to symptoms that simulate those of arteriosclerosis.</p><p id="par0060" class="elsevierStylePara elsevierViewall">In summary, although any calcification observed on mammography should be an alarm signal, it is important to know the frequency of association of mammographic microcalcifications with PXE and to take this into account in the differential diagnosis of breast cancer.<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">7,8</span></a></p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara">Please cite this article as: Padilla-España L, Fernández-Morano T, Del Boz J, Fúnez R. Seudoxantoma elástico en el diagnóstico diferencial de las calcificaciones axilares en la mamografía. Actas Dermosifiliogr.2012;103:647-649.</p>" ] ] "multimedia" => array:3 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1986 "Ancho" => 951 "Tamanyo" => 210056 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Mammography of the left breast.</p>" ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 530 "Ancho" => 1401 "Tamanyo" => 96182 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">A, Redundant skin folds in the right axilla. B, Redundant skin folds in the left axilla.</p>" ] ] 2 => array:7 [ "identificador" => "fig0015" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 543 "Ancho" => 1401 "Tamanyo" => 352665 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">A, Orcein stain, original magnification<span class="elsevierStyleHsp" style=""></span>×100. B, von Kossa stain, original magnification<span class="elsevierStyleHsp" style=""></span>×200.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:8 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Mapping of both autosomal recessive and dominant variants of pseudoxanthoma elasticum to chromosome 16p131" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:5 [ 0 => "B. Struck" 1 => "K.H. Neldner" 2 => "V. Rao" 3 => "P. St Jean" 4 => "K. 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| año/Mes | Html | Total | |
|---|---|---|---|
| 2024 Noviembre | 7 | 15 | 22 |
| 2024 Octubre | 74 | 47 | 121 |
| 2024 Septiembre | 79 | 54 | 133 |
| 2024 Agosto | 112 | 93 | 205 |
| 2024 Julio | 97 | 55 | 152 |
| 2024 Junio | 107 | 70 | 177 |
| 2024 Mayo | 82 | 46 | 128 |
| 2024 Abril | 77 | 31 | 108 |
| 2024 Marzo | 99 | 44 | 143 |
| 2024 Febrero | 79 | 40 | 119 |
| 2024 Enero | 71 | 44 | 115 |
| 2023 Diciembre | 73 | 29 | 102 |
| 2023 Noviembre | 65 | 37 | 102 |
| 2023 Octubre | 71 | 19 | 90 |
| 2023 Septiembre | 67 | 38 | 105 |
| 2023 Agosto | 60 | 37 | 97 |
| 2023 Julio | 72 | 62 | 134 |
| 2023 Junio | 68 | 45 | 113 |
| 2023 Mayo | 83 | 56 | 139 |
| 2023 Abril | 59 | 31 | 90 |
| 2023 Marzo | 66 | 49 | 115 |
| 2023 Febrero | 76 | 26 | 102 |
| 2023 Enero | 49 | 40 | 89 |
| 2022 Diciembre | 63 | 35 | 98 |
| 2022 Noviembre | 37 | 32 | 69 |
| 2022 Octubre | 35 | 47 | 82 |
| 2022 Septiembre | 25 | 46 | 71 |
| 2022 Agosto | 24 | 34 | 58 |
| 2022 Julio | 29 | 35 | 64 |
| 2022 Junio | 32 | 43 | 75 |
| 2022 Mayo | 45 | 41 | 86 |
| 2022 Abril | 71 | 38 | 109 |
| 2022 Marzo | 79 | 54 | 133 |
| 2022 Febrero | 66 | 29 | 95 |
| 2022 Enero | 58 | 56 | 114 |
| 2021 Diciembre | 56 | 51 | 107 |
| 2021 Noviembre | 42 | 62 | 104 |
| 2021 Octubre | 57 | 65 | 122 |
| 2021 Septiembre | 38 | 65 | 103 |
| 2021 Agosto | 31 | 41 | 72 |
| 2021 Julio | 38 | 35 | 73 |
| 2021 Junio | 56 | 58 | 114 |
| 2021 Mayo | 37 | 64 | 101 |
| 2021 Abril | 85 | 129 | 214 |
| 2021 Marzo | 100 | 62 | 162 |
| 2021 Febrero | 61 | 35 | 96 |
| 2021 Enero | 63 | 28 | 91 |
| 2020 Diciembre | 57 | 26 | 83 |
| 2020 Noviembre | 36 | 22 | 58 |
| 2020 Octubre | 39 | 26 | 65 |
| 2020 Septiembre | 38 | 23 | 61 |
| 2020 Agosto | 39 | 31 | 70 |
| 2020 Julio | 33 | 17 | 50 |
| 2020 Junio | 40 | 35 | 75 |
| 2020 Mayo | 31 | 30 | 61 |
| 2020 Abril | 34 | 35 | 69 |
| 2020 Marzo | 35 | 21 | 56 |
| 2020 Febrero | 1 | 7 | 8 |
| 2020 Enero | 0 | 7 | 7 |
| 2019 Diciembre | 4 | 8 | 12 |
| 2019 Noviembre | 0 | 4 | 4 |
| 2019 Octubre | 0 | 1 | 1 |
| 2019 Septiembre | 3 | 2 | 5 |
| 2019 Julio | 0 | 4 | 4 |
| 2019 Junio | 2 | 1 | 3 |
| 2019 Mayo | 0 | 11 | 11 |
| 2019 Abril | 0 | 3 | 3 |
| 2019 Marzo | 2 | 10 | 12 |
| 2019 Febrero | 0 | 1 | 1 |
| 2019 Enero | 3 | 0 | 3 |
| 2018 Diciembre | 2 | 1 | 3 |
| 2018 Noviembre | 3 | 0 | 3 |
| 2018 Octubre | 4 | 0 | 4 |
| 2018 Septiembre | 4 | 2 | 6 |
| 2018 Agosto | 0 | 3 | 3 |
| 2018 Julio | 0 | 2 | 2 |
| 2018 Junio | 0 | 3 | 3 |
| 2018 Mayo | 0 | 5 | 5 |
| 2018 Abril | 0 | 2 | 2 |
| 2018 Marzo | 3 | 1 | 4 |
| 2018 Febrero | 85 | 8 | 93 |
| 2018 Enero | 55 | 7 | 62 |
| 2017 Diciembre | 99 | 7 | 106 |
| 2017 Noviembre | 40 | 3 | 43 |
| 2017 Octubre | 43 | 12 | 55 |
| 2017 Septiembre | 44 | 13 | 57 |
| 2017 Agosto | 56 | 21 | 77 |
| 2017 Julio | 34 | 11 | 45 |
| 2017 Junio | 53 | 31 | 84 |
| 2017 Mayo | 39 | 11 | 50 |
| 2017 Abril | 33 | 10 | 43 |
| 2017 Marzo | 32 | 33 | 65 |
| 2017 Febrero | 44 | 14 | 58 |
| 2017 Enero | 31 | 13 | 44 |
| 2016 Diciembre | 55 | 12 | 67 |
| 2016 Noviembre | 64 | 16 | 80 |
| 2016 Octubre | 98 | 24 | 122 |
| 2016 Septiembre | 127 | 10 | 137 |
| 2016 Agosto | 81 | 12 | 93 |
| 2016 Julio | 52 | 17 | 69 |
| 2016 Junio | 11 | 8 | 19 |
| 2016 Mayo | 6 | 14 | 20 |
| 2016 Abril | 1 | 9 | 10 |
| 2016 Marzo | 9 | 1 | 10 |
| 2016 Febrero | 7 | 1 | 8 |
| 2016 Enero | 10 | 2 | 12 |
| 2015 Diciembre | 5 | 7 | 12 |
| 2015 Noviembre | 23 | 11 | 34 |
| 2015 Octubre | 23 | 10 | 33 |
| 2015 Septiembre | 12 | 3 | 15 |
| 2015 Agosto | 8 | 3 | 11 |
| 2015 Julio | 148 | 9 | 157 |
| 2015 Junio | 76 | 6 | 82 |
| 2015 Mayo | 95 | 20 | 115 |
| 2015 Abril | 87 | 11 | 98 |
| 2015 Marzo | 88 | 10 | 98 |
| 2015 Febrero | 95 | 19 | 114 |
| 2015 Enero | 71 | 10 | 81 |
| 2014 Diciembre | 75 | 4 | 79 |
| 2014 Noviembre | 62 | 15 | 77 |
| 2014 Octubre | 69 | 15 | 84 |
| 2014 Septiembre | 78 | 9 | 87 |
| 2014 Agosto | 35 | 2 | 37 |
| 2014 Julio | 36 | 4 | 40 |
| 2014 Junio | 46 | 3 | 49 |
| 2014 Mayo | 48 | 6 | 54 |
| 2014 Abril | 34 | 3 | 37 |
| 2014 Marzo | 50 | 10 | 60 |
| 2014 Febrero | 41 | 11 | 52 |
| 2014 Enero | 46 | 7 | 53 |
| 2013 Diciembre | 48 | 11 | 59 |
| 2013 Noviembre | 33 | 6 | 39 |
| 2013 Octubre | 29 | 8 | 37 |
| 2013 Septiembre | 21 | 7 | 28 |
| 2013 Agosto | 11 | 10 | 21 |
| 2013 Julio | 11 | 15 | 26 |
| 2013 Junio | 7 | 21 | 28 |
| 2013 Mayo | 14 | 17 | 31 |
| 2013 Abril | 17 | 16 | 33 |
| 2013 Marzo | 20 | 10 | 30 |
| 2013 Febrero | 24 | 4 | 28 |
| 2013 Enero | 55 | 6 | 61 |
| 2012 Diciembre | 23 | 4 | 27 |
| 2012 Noviembre | 1 | 1 | 2 |
| 2012 Octubre | 0 | 3 | 3 |
| 2012 Septiembre | 0 | 1 | 1 |
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