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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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 8 | 15 | 23 |
| 2024 October | 74 | 47 | 121 |
| 2024 September | 79 | 54 | 133 |
| 2024 August | 112 | 93 | 205 |
| 2024 July | 97 | 55 | 152 |
| 2024 June | 107 | 70 | 177 |
| 2024 May | 82 | 46 | 128 |
| 2024 April | 77 | 31 | 108 |
| 2024 March | 99 | 44 | 143 |
| 2024 February | 79 | 40 | 119 |
| 2024 January | 71 | 44 | 115 |
| 2023 December | 73 | 29 | 102 |
| 2023 November | 65 | 37 | 102 |
| 2023 October | 71 | 19 | 90 |
| 2023 September | 67 | 38 | 105 |
| 2023 August | 60 | 37 | 97 |
| 2023 July | 72 | 62 | 134 |
| 2023 June | 68 | 45 | 113 |
| 2023 May | 83 | 56 | 139 |
| 2023 April | 59 | 31 | 90 |
| 2023 March | 66 | 49 | 115 |
| 2023 February | 76 | 26 | 102 |
| 2023 January | 49 | 40 | 89 |
| 2022 December | 63 | 35 | 98 |
| 2022 November | 37 | 32 | 69 |
| 2022 October | 35 | 47 | 82 |
| 2022 September | 25 | 46 | 71 |
| 2022 August | 24 | 34 | 58 |
| 2022 July | 29 | 35 | 64 |
| 2022 June | 32 | 43 | 75 |
| 2022 May | 45 | 41 | 86 |
| 2022 April | 71 | 38 | 109 |
| 2022 March | 79 | 54 | 133 |
| 2022 February | 66 | 29 | 95 |
| 2022 January | 58 | 56 | 114 |
| 2021 December | 56 | 51 | 107 |
| 2021 November | 42 | 62 | 104 |
| 2021 October | 57 | 65 | 122 |
| 2021 September | 38 | 65 | 103 |
| 2021 August | 31 | 41 | 72 |
| 2021 July | 38 | 35 | 73 |
| 2021 June | 56 | 58 | 114 |
| 2021 May | 37 | 64 | 101 |
| 2021 April | 85 | 129 | 214 |
| 2021 March | 100 | 62 | 162 |
| 2021 February | 61 | 35 | 96 |
| 2021 January | 63 | 28 | 91 |
| 2020 December | 57 | 26 | 83 |
| 2020 November | 36 | 22 | 58 |
| 2020 October | 39 | 26 | 65 |
| 2020 September | 38 | 23 | 61 |
| 2020 August | 39 | 31 | 70 |
| 2020 July | 33 | 17 | 50 |
| 2020 June | 40 | 35 | 75 |
| 2020 May | 31 | 30 | 61 |
| 2020 April | 34 | 35 | 69 |
| 2020 March | 35 | 21 | 56 |
| 2020 February | 1 | 7 | 8 |
| 2020 January | 0 | 7 | 7 |
| 2019 December | 4 | 8 | 12 |
| 2019 November | 0 | 4 | 4 |
| 2019 October | 0 | 1 | 1 |
| 2019 September | 3 | 2 | 5 |
| 2019 July | 0 | 4 | 4 |
| 2019 June | 2 | 1 | 3 |
| 2019 May | 0 | 11 | 11 |
| 2019 April | 0 | 3 | 3 |
| 2019 March | 2 | 10 | 12 |
| 2019 February | 0 | 1 | 1 |
| 2019 January | 3 | 0 | 3 |
| 2018 December | 2 | 1 | 3 |
| 2018 November | 3 | 0 | 3 |
| 2018 October | 4 | 0 | 4 |
| 2018 September | 4 | 2 | 6 |
| 2018 August | 0 | 3 | 3 |
| 2018 July | 0 | 2 | 2 |
| 2018 June | 0 | 3 | 3 |
| 2018 May | 0 | 5 | 5 |
| 2018 April | 0 | 2 | 2 |
| 2018 March | 3 | 1 | 4 |
| 2018 February | 85 | 8 | 93 |
| 2018 January | 55 | 7 | 62 |
| 2017 December | 99 | 7 | 106 |
| 2017 November | 40 | 3 | 43 |
| 2017 October | 43 | 12 | 55 |
| 2017 September | 44 | 13 | 57 |
| 2017 August | 56 | 21 | 77 |
| 2017 July | 34 | 11 | 45 |
| 2017 June | 53 | 31 | 84 |
| 2017 May | 39 | 11 | 50 |
| 2017 April | 33 | 10 | 43 |
| 2017 March | 32 | 33 | 65 |
| 2017 February | 44 | 14 | 58 |
| 2017 January | 31 | 13 | 44 |
| 2016 December | 55 | 12 | 67 |
| 2016 November | 64 | 16 | 80 |
| 2016 October | 98 | 24 | 122 |
| 2016 September | 127 | 10 | 137 |
| 2016 August | 81 | 12 | 93 |
| 2016 July | 52 | 17 | 69 |
| 2016 June | 11 | 8 | 19 |
| 2016 May | 6 | 14 | 20 |
| 2016 April | 1 | 9 | 10 |
| 2016 March | 9 | 1 | 10 |
| 2016 February | 7 | 1 | 8 |
| 2016 January | 10 | 2 | 12 |
| 2015 December | 5 | 7 | 12 |
| 2015 November | 23 | 11 | 34 |
| 2015 October | 23 | 10 | 33 |
| 2015 September | 12 | 3 | 15 |
| 2015 August | 8 | 3 | 11 |
| 2015 July | 148 | 9 | 157 |
| 2015 June | 76 | 6 | 82 |
| 2015 May | 95 | 20 | 115 |
| 2015 April | 87 | 11 | 98 |
| 2015 March | 88 | 10 | 98 |
| 2015 February | 95 | 19 | 114 |
| 2015 January | 71 | 10 | 81 |
| 2014 December | 75 | 4 | 79 |
| 2014 November | 62 | 15 | 77 |
| 2014 October | 69 | 15 | 84 |
| 2014 September | 78 | 9 | 87 |
| 2014 August | 35 | 2 | 37 |
| 2014 July | 36 | 4 | 40 |
| 2014 June | 46 | 3 | 49 |
| 2014 May | 48 | 6 | 54 |
| 2014 April | 34 | 3 | 37 |
| 2014 March | 50 | 10 | 60 |
| 2014 February | 41 | 11 | 52 |
| 2014 January | 46 | 7 | 53 |
| 2013 December | 48 | 11 | 59 |
| 2013 November | 33 | 6 | 39 |
| 2013 October | 29 | 8 | 37 |
| 2013 September | 21 | 7 | 28 |
| 2013 August | 11 | 10 | 21 |
| 2013 July | 11 | 15 | 26 |
| 2013 June | 7 | 21 | 28 |
| 2013 May | 14 | 17 | 31 |
| 2013 April | 17 | 16 | 33 |
| 2013 March | 20 | 10 | 30 |
| 2013 February | 24 | 4 | 28 |
| 2013 January | 55 | 6 | 61 |
| 2012 December | 23 | 4 | 27 |
| 2012 November | 1 | 1 | 2 |
| 2012 October | 0 | 3 | 3 |
| 2012 September | 0 | 1 | 1 |
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