Case for Diagnosis
Reticulate Hyperpigmentation of the Flexures
Hiperpigmentación reticulada en pliegues
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"apellidos" => "García-Donoso" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Servicio de Dermatología, Hospital Universitario de Fuenlabrada, Fuenlabrada, Madrid, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Anatomía Patológica, Hospital Universitario de Fuenlabrada, Fuenlabrada, Madrid, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Hiperpigmentación reticulada en pliegues" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0015" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 652 "Ancho" => 815 "Tamanyo" => 147659 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Hematoxylin and eosin, original magnification<span class="elsevierStyleHsp" style=""></span>×40.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Medical History</span><p id="par0005" class="elsevierStylePara elsevierViewall">A 42-year-old woman with a past history of subclinical hypothyroidism of autoimmune origin, consulted for skin lesions that had developed progressively over 3 years in the axillas and groin. She complained about the past occasional pruritus with sweating. The patient was not aware of any similar lesions among her relatives.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Physical Examination</span><p id="par0010" class="elsevierStylePara elsevierViewall">Grayish-brown macules measuring a few millimeters in diameter were observed in both axillas (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). The lesions were not infiltrated and in some areas they were grouped in a reticulate pattern a reticulate. Similar lesions were observed in the groin (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>). The rest of the physical examination, including the skin adnexa and mucosas, revealed no relevant findings.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Histopathology</span><p id="par0015" class="elsevierStylePara elsevierViewall">A biopsy of one of the lesions showed mild hyperkeratosis of the epidermis, with elongation and hyperpigmentation of the rete ridges, occasional small corneal cysts, and the presence of melanophages in the superficial dermis (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>).</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleBold">What Is Your Diagnosis?</span></p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Diagnosis</span><p id="par0025" class="elsevierStylePara elsevierViewall">Dowling-Degos disease (DDD).</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Clinical Course and Treatment</span><p id="par0030" class="elsevierStylePara elsevierViewall">The patient was discharged with no treatment.</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Comment</span><p id="par0035" class="elsevierStylePara elsevierViewall">DDD or reticular pigmented anomaly of the flexures is a rare genodermatosis of autosomal dominant inheritance with variable penetrance. Manifestations arise between 30 and 40 years of age. It was described by Behçet as a variant of acanthosis nigricans, from which it was differentiated by Dowling and Freudenthal in 1938; it was called <span class="elsevierStyleItalic">dermatose pigmentaire réticulée des plis</span> by Degos and Ossipowski, and finally named Dowling-Degos disease in 1978 by Jones and Grice.</p><p id="par0040" class="elsevierStylePara elsevierViewall">Genetic alterations described in DDD include mutation and loss of function of the keratin 5 gene,<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a> changes at chromosome 17p13.3, and mutations of <span class="elsevierStyleItalic">POFUT1</span><a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a> and <span class="elsevierStyleItalic">POGLUT1</span>,<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a> which encode the proteins O-fucosyltransferase<span class="elsevierStyleHsp" style=""></span>1 and O-glucosyltransferase<span class="elsevierStyleHsp" style=""></span>1, implicated in the NOTCH pathway.</p><p id="par0045" class="elsevierStylePara elsevierViewall">The disease presents as hyperpigmented macules with a reticulate pattern in the axillas and groin. The macules can spread to other skin folds and may be associated with comedo-like lesions on the back and neck, pitted pinpoint perioral scars, epidermal cysts, hidradenitis suppurativa, squamous cell carcinomas, and multiple keratoacanthomas.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a> Histopathology shows orthokeratosis or hyperkeratosis, thinning of the suprapapillary epithelium, elongation of the rete ridges, hyperpigmentation of the basal layer, and follicular cysts. A perivascular lymphohistiocytic infiltrate can be observed in the papillary dermis.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a> The differential diagnosis should include acanthosis nigricans and granular parakeratosis, as well as various entities considered by some authors to be part of the spectrum of a single disease: Galli-Galli disease (GGD), reticulate acropigmentation of Kitamura (RAK), Haber syndrome (HS), reticulate acropigmentation of Dohi (ARD) or dyschromatosis symmetrica hereditaria (DSH), and dyschromatosis universalis hereditaria (DUH).<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a> In addition to the findings described for DDD, we also find atrophic acral hyperpigmented macules and palmar pitting or fissures in RAK, rosacea-like facial erythema and hyperkeratotic pigmented papules on the trunk in HS, and hyperpigmented and hypopigmented macules on the dorsum of the hands and feet in DSH or ARD and widespread in DUH. DDD and GGD are indistinguishable based on clinical findings and are differentiated by the presence of acantholysis on histopathology.<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">5</span></a> The later onset and the absence of other clinical findings will differentiate DDD from other diseases that cause early-onset reticulate hyperpigmentation: Naegeli-Franceschetti-Jadassohn syndrome, congenital dyskeratosis, and dermopathia pigmentosa reticularis.</p><p id="par0050" class="elsevierStylePara elsevierViewall">From a histologic point of view, DDD must be differentiated from acanthosis nigricans and seborrheic keratosis with an adenoid pattern. Involvement of the infundibulum of the hair follicle is a characteristic finding in DDD but is not seen in the other 2 conditions.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">6</span></a> DDD has been treated with hydroquinone, adapalene, tretinoin, and topical corticosteroids, with variable results. Another option is the Er: YAG laser treatment.</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Conflicts of interest</span><p id="par0055" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:9 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Medical History" ] 1 => array:2 [ "identificador" => "sec0010" "titulo" => "Physical Examination" ] 2 => array:2 [ "identificador" => "sec0015" "titulo" => "Histopathology" ] 3 => array:2 [ "identificador" => "sec0020" "titulo" => "Diagnosis" ] 4 => array:2 [ "identificador" => "sec0025" "titulo" => "Clinical Course and Treatment" ] 5 => array:2 [ "identificador" => "sec0030" "titulo" => "Comment" ] 6 => array:2 [ "identificador" => "sec0035" "titulo" => "Conflicts of interest" ] 7 => array:2 [ "identificador" => "xack253808" "titulo" => "Acknowledgments" ] 8 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: González-Olivares M, Nájera L, García-Donoso C. Hiperpigmentación reticulada en pliegues. Actas Dermosifiliogr. 2016;107:853–854.</p>" ] ] "multimedia" => array:3 [ 0 => array:6 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 354 "Ancho" => 950 "Tamanyo" => 65241 ] ] ] 1 => array:6 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 600 "Ancho" => 854 "Tamanyo" => 87126 ] ] ] 2 => array:7 [ "identificador" => "fig0015" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 652 "Ancho" => 815 "Tamanyo" => 147659 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Hematoxylin and eosin, original magnification<span class="elsevierStyleHsp" style=""></span>×40.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:6 [ 0 => array:3 [ "identificador" => "bib0035" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Loss-of-function mutations in the keratin 5 gene lead to Dowling-Degos disease" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "R.C. 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| año/Mes | Html | Total | |
|---|---|---|---|
| 2024 Noviembre | 12 | 9 | 21 |
| 2024 Octubre | 112 | 38 | 150 |
| 2024 Septiembre | 106 | 21 | 127 |
| 2024 Agosto | 123 | 51 | 174 |
| 2024 Julio | 104 | 27 | 131 |
| 2024 Junio | 117 | 39 | 156 |
| 2024 Mayo | 92 | 28 | 120 |
| 2024 Abril | 101 | 27 | 128 |
| 2024 Marzo | 90 | 18 | 108 |
| 2024 Febrero | 74 | 38 | 112 |
| 2024 Enero | 80 | 30 | 110 |
| 2023 Diciembre | 79 | 15 | 94 |
| 2023 Noviembre | 82 | 29 | 111 |
| 2023 Octubre | 87 | 12 | 99 |
| 2023 Septiembre | 100 | 29 | 129 |
| 2023 Agosto | 72 | 13 | 85 |
| 2023 Julio | 95 | 24 | 119 |
| 2023 Junio | 92 | 30 | 122 |
| 2023 Mayo | 69 | 21 | 90 |
| 2023 Abril | 69 | 16 | 85 |
| 2023 Marzo | 78 | 20 | 98 |
| 2023 Febrero | 75 | 19 | 94 |
| 2023 Enero | 68 | 34 | 102 |
| 2022 Diciembre | 99 | 31 | 130 |
| 2022 Noviembre | 42 | 25 | 67 |
| 2022 Octubre | 36 | 24 | 60 |
| 2022 Septiembre | 37 | 33 | 70 |
| 2022 Agosto | 38 | 29 | 67 |
| 2022 Julio | 33 | 35 | 68 |
| 2022 Junio | 41 | 21 | 62 |
| 2022 Mayo | 52 | 31 | 83 |
| 2022 Abril | 61 | 27 | 88 |
| 2022 Marzo | 60 | 49 | 109 |
| 2022 Febrero | 48 | 26 | 74 |
| 2022 Enero | 67 | 35 | 102 |
| 2021 Diciembre | 49 | 30 | 79 |
| 2021 Noviembre | 46 | 52 | 98 |
| 2021 Octubre | 71 | 52 | 123 |
| 2021 Septiembre | 69 | 46 | 115 |
| 2021 Agosto | 51 | 46 | 97 |
| 2021 Julio | 46 | 22 | 68 |
| 2021 Junio | 53 | 38 | 91 |
| 2021 Mayo | 70 | 43 | 113 |
| 2021 Abril | 142 | 52 | 194 |
| 2021 Marzo | 76 | 25 | 101 |
| 2021 Febrero | 63 | 37 | 100 |
| 2021 Enero | 39 | 14 | 53 |
| 2020 Diciembre | 35 | 15 | 50 |
| 2020 Noviembre | 32 | 15 | 47 |
| 2020 Octubre | 39 | 6 | 45 |
| 2020 Septiembre | 22 | 13 | 35 |
| 2020 Agosto | 26 | 12 | 38 |
| 2020 Julio | 29 | 13 | 42 |
| 2020 Junio | 32 | 33 | 65 |
| 2020 Mayo | 19 | 9 | 28 |
| 2020 Abril | 25 | 13 | 38 |
| 2020 Marzo | 17 | 15 | 32 |
| 2020 Febrero | 3 | 0 | 3 |
| 2019 Diciembre | 4 | 0 | 4 |
| 2019 Septiembre | 4 | 0 | 4 |
| 2019 Junio | 2 | 0 | 2 |
| 2019 Abril | 0 | 5 | 5 |
| 2019 Marzo | 3 | 0 | 3 |
| 2019 Febrero | 2 | 0 | 2 |
| 2019 Enero | 2 | 0 | 2 |
| 2018 Diciembre | 6 | 0 | 6 |
| 2018 Noviembre | 3 | 0 | 3 |
| 2018 Octubre | 2 | 0 | 2 |
| 2018 Septiembre | 6 | 0 | 6 |
| 2018 Febrero | 34 | 2 | 36 |
| 2018 Enero | 125 | 17 | 142 |
| 2017 Diciembre | 47 | 8 | 55 |
| 2017 Noviembre | 32 | 3 | 35 |
| 2017 Octubre | 40 | 8 | 48 |
| 2017 Septiembre | 33 | 4 | 37 |
| 2017 Agosto | 36 | 7 | 43 |
| 2017 Julio | 33 | 8 | 41 |
| 2017 Junio | 45 | 5 | 50 |
| 2017 Mayo | 23 | 6 | 29 |
| 2017 Abril | 42 | 13 | 55 |
| 2017 Marzo | 23 | 17 | 40 |
| 2017 Febrero | 27 | 6 | 33 |
| 2017 Enero | 17 | 8 | 25 |
| 2016 Diciembre | 66 | 37 | 103 |
| 2016 Noviembre | 20 | 25 | 45 |
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