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B, Light-brownish areas tending to converge; multiple linear and branching vessels can be seen. C, Nearly total absence of elastic fibers in the papillary dermis; in contrast, elastic fibers (black arrows) can be seen in the reticular dermis (orcein, original magnification ×100). D, Melanophages (red arrows) dispersed in the papillary dermis (hematoxilin-eosin ×400). E, Von Kossa staining revealed no calcifications of elastic fibers (original magnification ×<span class="elsevierStyleHsp" style=""></span>100).</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "I. Vázquez-Osorio, E. Rosón, J.M. Suárez-Peñaranda, H. Vázquez-Veiga" "autores" => array:4 [ 0 => array:2 [ "nombre" => "I." "apellidos" => "Vázquez-Osorio" ] 1 => array:2 [ "nombre" => "E." "apellidos" => "Rosón" ] 2 => array:2 [ "nombre" => "J.M." "apellidos" => "Suárez-Peñaranda" ] 3 => array:2 [ "nombre" => "H." 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Arjona-Aguilera, M. Linares-Barrios, C. Albarrán-Planelles, D. Jiménez-Gallo" "autores" => array:4 [ 0 => array:4 [ "nombre" => "C." "apellidos" => "Arjona-Aguilera" "email" => array:1 [ 0 => "cintiaarjona@hotmail.com" ] "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:2 [ "nombre" => "M." "apellidos" => "Linares-Barrios" ] 2 => array:2 [ "nombre" => "C." "apellidos" => "Albarrán-Planelles" ] 3 => array:2 [ "nombre" => "D." "apellidos" => "Jiménez-Gallo" ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Servicio de Dermatología Médico-Quirúrgica y Venereología, Hospital Universitario Puerta del Mar, Cádiz, Spain" "identificador" => "aff0005" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Enfermedad de Dowling-Degos: caso clínico asociado a hidradenitis supurativa" ] ] "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" => 747 "Ancho" => 996 "Tamanyo" => 153851 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Axillary nodules with a seropurulent exudate and scarred appearance, compatible with hidradenitis suppurativa.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Dowling-Degos disease (DDD) or reticular pigmented anomaly of the flexures is a rare autosomal dominant genodermatosis with variable penetrance.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1–3</span></a> It mainly affects young women and is characterized by acquired reticular hyperpigmentation of the large skin folds. We present a case of DDD associated with hidradenitis suppurativa (HS) in a 43-year-old Spanish woman. Physical examination revealed brownish macules of 5-10<span class="elsevierStyleHsp" style=""></span>mm diameter, with an irregular morphology, homogeneous pigmentation, and a reticular pattern, situated predominantly in the posterior cervical region (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>A) and inguinal and intergluteal folds (Fig.<span class="elsevierStyleHsp" style=""></span>1B). In addition, she presented active HS lesions in both axillas (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>), as well as depressed pinpoint perioral scars suggestive of acne. The patient stated that her mother, 2 maternal uncles, and 1 of her sisters had similar lesions.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">The dermatopathology study of a perianal skin lesion revealed basal hyperpigmentation, thin elongated digiform rete ridges, and suprapapillary thinning, as well as dermal melanophages and a mild perivascular lymphohistiocytic infiltrate (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>).</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Based on these data, we made a diagnosis of DDD with associated HS. We offered topical therapy as the first option, but this was rejected by the patient. The disease has shown no significant change after more than 5<span class="elsevierStyleHsp" style=""></span>years of follow-up.</p><p id="par0020" class="elsevierStylePara elsevierViewall">DDD is a rare autosomal dominant genodermatosis that was first described by Behçet in 1932 as a variant of acanthosis nigricans of the axillas. Since then a number of cases of DDD have been reported, and there have been further revelations about its etiology, pathogenesis, and treatment.</p><p id="par0025" class="elsevierStylePara elsevierViewall">DDD usually develops after puberty and tends to have a progressive course.<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">2,3</span></a> It is characterized clinically by brownish hyperpigmented macules that adopt a reticular pattern. These macules are situated in the skin flexures (submammary, axillas, groin), cervical region, trunk, and anterior surface of the thighs and upper arms. The presence of pinpoint papules with keratin plugs simulating comedones is also common in the palmar, axillary, cervical, perioral, and gluteal regions. Furthermore, depressed perioral scars can develop,<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">2,3</span></a> as was observed in our case.</p><p id="par0030" class="elsevierStylePara elsevierViewall">Dermatologic histopathology is characteristic, showing interconnected hyperpigmented epidermal proliferations projecting in a filiform pattern into the dermis. This is called the “antler-like” pattern. Basal-layer melanocytes are present in normal numbers. Epidermal hyperplasia may be observed in the hair follicles, extending into the dermis, with the formation of plugs and follicular dilation. The other histologic feature is a mild perivascular lymphohistiocytic infiltrate. The key feature that differentiates DDD from other disorders of keratinization is the combination of hyperpigmented proliferations derived both from the epidermis and from the follicular wall.</p><p id="par0035" class="elsevierStylePara elsevierViewall">The etiology and pathogenesis of DDD involve mutations that have been shown to produce haploinsufficiency of the keratin-5 gene (<span class="elsevierStyleItalic">KRT5</span>) on chromosome<span class="elsevierStyleHsp" style=""></span>12q.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">3</span></a> Keratin-5 is an essential element of the basal keratinocyte cytoskeleton, together with keratin-14. Keratin-5 dysfunction leads to alterations in organelle transport and in epidermal differentiation. In addition, a study in 2013 identified a mutation in chromosome<span class="elsevierStyleHsp" style=""></span>20 in a family with generalized DDD.<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">4</span></a> The heterozygous deletion of gene <span class="elsevierStyleItalic">POFUT1</span> leads to decreased expression of keratin<span class="elsevierStyleHsp" style=""></span>5 and other proteins (Notch1-2, Hes1) in keratinocytes.</p><p id="par0040" class="elsevierStylePara elsevierViewall">DDD has been associated with certain skin diseases, in particular multiple keratoacanthomas,<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">5</span></a> epidermoid cysts,<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">5</span></a> and squamous cell carcinoma.<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">6</span></a> An important association is with HS, as found in our patient, though few cases have been reported in the literature.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1,2,5,7–9</span></a> HS is a multifactorial disease that most commonly affects young people and is characterized by recurrent skin-fold abscesses and fistulas that leave scars. One etiologic and pathogenic factor that has been observed is a defect of epithelial proliferation in the external sheath, leading to follicular occlusion, described as a possible common origin for all these disorders.<a class="elsevierStyleCrossRefs" href="#bib0075"><span class="elsevierStyleSup">5,7,9</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">DDD must be differentiated from other genodermatoses with reticular pigmentation<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">2,3</span></a>: Galli-Galli disease,<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">10</span></a> reticulate acropigmentation of Kitamura, Haber syndrome, and reticulate acropigmentation of Dohi. It has recently been agreed that all these reticular pigmentary diseases are variants of a single entity.<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">5</span></a> Other important diseases in the differential diagnosis include acanthosis nigricans, Naegeli-Franceschetti-Jadassohn syndrome, and confluent and reticulate papillomatosis (Gougerot-Carteaud syndrome).</p><p id="par0050" class="elsevierStylePara elsevierViewall">There is no completely effective treatment for DDD. Topical hydroquinone, retinoids, and corticosteroids, oral isotretinoin, and some lasers have been used with variable results. Topical adapalene and tazarotene have been the most successful of the treatments used. This derives from their anti-inflammatory properties as well as their modulating action on gene expression and on keratinocyte differentiation and proliferation. Several types of laser, in particular carbon dioxide and erbium-doped yttrium aluminium garnet (Er:YAG) lasers, have been shown to be useful in reducing or eliminating the hyperpigmentation.</p><p id="par0055" class="elsevierStylePara elsevierViewall">In conclusion, we have presented a case of DDD associated with HS, a previously reported but rare association. The lesions are progressive and recurrent. Topical and oral retinoids are a safe and accessible treatment option, though not particularly effective. It is important to maintain long-term follow-up in these patients due to the possible association with skin tumors.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Arjona-Aguilera C, Linares-Barrios M, Albarrán-Planelles C, Jiménez-Gallo D. Enfermedad de Dowling-Degos: caso clínico asociado a hidradenitis supurativa. Actas Dermosifiliogr. 2015;106:337–338.</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" => 626 "Ancho" => 1299 "Tamanyo" => 117965 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Small brownish macules with a tendency to coalesce in a reticular pattern. A, In the posterior cervical region. B, In the intergluteal fold.</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" => 747 "Ancho" => 996 "Tamanyo" => 153851 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Axillary nodules with a seropurulent exudate and scarred appearance, compatible with hidradenitis suppurativa.</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" => 746 "Ancho" => 995 "Tamanyo" => 323119 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Histopathology of a lesion biopsied from the perianal skin: basal hyperpigmentation, digitiform rete ridges, antler-like suprapapillary thinning, dermal melanophages, and a mild perivascular lymphohistiocytic infiltrate (hematoxylin and eosin, original magnification ×<span class="elsevierStyleHsp" style=""></span>10).</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:10 [ 0 => array:3 [ "identificador" => "bib0055" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Anomalía pigmentada y reticular de las flexuras (enfermedad de Dowling-Degos)" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "I. 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año/Mes | Html | Total | |
---|---|---|---|
2024 Noviembre | 8 | 8 | 16 |
2024 Octubre | 97 | 44 | 141 |
2024 Septiembre | 110 | 19 | 129 |
2024 Agosto | 128 | 56 | 184 |
2024 Julio | 137 | 31 | 168 |
2024 Junio | 118 | 39 | 157 |
2024 Mayo | 119 | 51 | 170 |
2024 Abril | 93 | 31 | 124 |
2024 Marzo | 111 | 41 | 152 |
2024 Febrero | 93 | 44 | 137 |
2024 Enero | 132 | 77 | 209 |
2023 Diciembre | 80 | 31 | 111 |
2023 Noviembre | 145 | 26 | 171 |
2023 Octubre | 145 | 26 | 171 |
2023 Septiembre | 169 | 40 | 209 |
2023 Agosto | 100 | 24 | 124 |
2023 Julio | 133 | 74 | 207 |
2023 Junio | 114 | 31 | 145 |
2023 Mayo | 202 | 61 | 263 |
2023 Abril | 157 | 42 | 199 |
2023 Marzo | 129 | 50 | 179 |
2023 Febrero | 111 | 33 | 144 |
2023 Enero | 107 | 42 | 149 |
2022 Diciembre | 81 | 52 | 133 |
2022 Noviembre | 43 | 27 | 70 |
2022 Octubre | 51 | 25 | 76 |
2022 Septiembre | 86 | 43 | 129 |
2022 Agosto | 54 | 46 | 100 |
2022 Julio | 43 | 33 | 76 |
2022 Junio | 49 | 29 | 78 |
2022 Mayo | 99 | 44 | 143 |
2022 Abril | 123 | 43 | 166 |
2022 Marzo | 84 | 39 | 123 |
2022 Febrero | 101 | 28 | 129 |
2022 Enero | 96 | 44 | 140 |
2021 Diciembre | 78 | 32 | 110 |
2021 Noviembre | 80 | 49 | 129 |
2021 Octubre | 119 | 53 | 172 |
2021 Septiembre | 82 | 42 | 124 |
2021 Agosto | 136 | 39 | 175 |
2021 Julio | 108 | 37 | 145 |
2021 Junio | 81 | 36 | 117 |
2021 Mayo | 84 | 26 | 110 |
2021 Abril | 182 | 66 | 248 |
2021 Marzo | 133 | 33 | 166 |
2021 Febrero | 120 | 33 | 153 |
2021 Enero | 70 | 24 | 94 |
2020 Diciembre | 81 | 22 | 103 |
2020 Noviembre | 53 | 23 | 76 |
2020 Octubre | 62 | 14 | 76 |
2020 Septiembre | 77 | 13 | 90 |
2020 Agosto | 31 | 19 | 50 |
2020 Julio | 31 | 18 | 49 |
2020 Junio | 46 | 30 | 76 |
2020 Mayo | 30 | 27 | 57 |
2020 Abril | 37 | 28 | 65 |
2020 Marzo | 39 | 20 | 59 |
2020 Febrero | 8 | 5 | 13 |
2020 Enero | 0 | 2 | 2 |
2019 Diciembre | 3 | 6 | 9 |
2019 Noviembre | 4 | 6 | 10 |
2019 Octubre | 1 | 2 | 3 |
2019 Septiembre | 8 | 8 | 16 |
2019 Agosto | 4 | 0 | 4 |
2019 Julio | 4 | 5 | 9 |
2019 Junio | 4 | 7 | 11 |
2019 Mayo | 4 | 18 | 22 |
2019 Abril | 2 | 10 | 12 |
2019 Marzo | 2 | 6 | 8 |
2019 Febrero | 4 | 0 | 4 |
2019 Enero | 1 | 0 | 1 |
2018 Diciembre | 2 | 0 | 2 |
2018 Noviembre | 1 | 6 | 7 |
2018 Octubre | 3 | 0 | 3 |
2018 Septiembre | 8 | 0 | 8 |
2018 Mayo | 0 | 3 | 3 |
2018 Abril | 0 | 2 | 2 |
2018 Marzo | 5 | 7 | 12 |
2018 Febrero | 30 | 6 | 36 |
2018 Enero | 42 | 4 | 46 |
2017 Diciembre | 39 | 11 | 50 |
2017 Noviembre | 34 | 8 | 42 |
2017 Octubre | 33 | 11 | 44 |
2017 Septiembre | 26 | 8 | 34 |
2017 Agosto | 32 | 6 | 38 |
2017 Julio | 30 | 19 | 49 |
2017 Junio | 20 | 6 | 26 |
2017 Mayo | 22 | 15 | 37 |
2017 Abril | 22 | 10 | 32 |
2017 Marzo | 24 | 23 | 47 |
2017 Febrero | 26 | 15 | 41 |
2017 Enero | 17 | 6 | 23 |
2016 Diciembre | 29 | 14 | 43 |
2016 Noviembre | 45 | 15 | 60 |
2016 Octubre | 37 | 20 | 57 |
2016 Septiembre | 0 | 10 | 10 |
2016 Agosto | 1 | 6 | 7 |
2016 Julio | 10 | 3 | 13 |
2016 Junio | 15 | 1 | 16 |
2016 Mayo | 15 | 1 | 16 |
2016 Abril | 8 | 3 | 11 |
2016 Marzo | 10 | 3 | 13 |
2016 Febrero | 8 | 2 | 10 |
2016 Enero | 10 | 6 | 16 |
2015 Diciembre | 13 | 10 | 23 |
2015 Noviembre | 10 | 9 | 19 |
2015 Octubre | 1 | 3 | 4 |
2015 Septiembre | 0 | 1 | 1 |
2015 Agosto | 0 | 2 | 2 |
2015 Julio | 10 | 1 | 11 |
2015 Junio | 3 | 6 | 9 |
2015 Mayo | 15 | 7 | 22 |