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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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 12 | 9 | 21 |
| 2024 October | 112 | 38 | 150 |
| 2024 September | 106 | 21 | 127 |
| 2024 August | 123 | 51 | 174 |
| 2024 July | 104 | 27 | 131 |
| 2024 June | 117 | 39 | 156 |
| 2024 May | 92 | 28 | 120 |
| 2024 April | 101 | 27 | 128 |
| 2024 March | 90 | 18 | 108 |
| 2024 February | 74 | 38 | 112 |
| 2024 January | 80 | 30 | 110 |
| 2023 December | 79 | 15 | 94 |
| 2023 November | 82 | 29 | 111 |
| 2023 October | 87 | 12 | 99 |
| 2023 September | 100 | 29 | 129 |
| 2023 August | 72 | 13 | 85 |
| 2023 July | 95 | 24 | 119 |
| 2023 June | 92 | 30 | 122 |
| 2023 May | 69 | 21 | 90 |
| 2023 April | 69 | 16 | 85 |
| 2023 March | 78 | 20 | 98 |
| 2023 February | 75 | 19 | 94 |
| 2023 January | 68 | 34 | 102 |
| 2022 December | 99 | 31 | 130 |
| 2022 November | 42 | 25 | 67 |
| 2022 October | 36 | 24 | 60 |
| 2022 September | 37 | 33 | 70 |
| 2022 August | 38 | 29 | 67 |
| 2022 July | 33 | 35 | 68 |
| 2022 June | 41 | 21 | 62 |
| 2022 May | 52 | 31 | 83 |
| 2022 April | 61 | 27 | 88 |
| 2022 March | 60 | 49 | 109 |
| 2022 February | 48 | 26 | 74 |
| 2022 January | 67 | 35 | 102 |
| 2021 December | 49 | 30 | 79 |
| 2021 November | 46 | 52 | 98 |
| 2021 October | 71 | 52 | 123 |
| 2021 September | 69 | 46 | 115 |
| 2021 August | 51 | 46 | 97 |
| 2021 July | 46 | 22 | 68 |
| 2021 June | 53 | 38 | 91 |
| 2021 May | 70 | 43 | 113 |
| 2021 April | 142 | 52 | 194 |
| 2021 March | 76 | 25 | 101 |
| 2021 February | 63 | 37 | 100 |
| 2021 January | 39 | 14 | 53 |
| 2020 December | 35 | 15 | 50 |
| 2020 November | 32 | 15 | 47 |
| 2020 October | 39 | 6 | 45 |
| 2020 September | 22 | 13 | 35 |
| 2020 August | 26 | 12 | 38 |
| 2020 July | 29 | 13 | 42 |
| 2020 June | 32 | 33 | 65 |
| 2020 May | 19 | 9 | 28 |
| 2020 April | 25 | 13 | 38 |
| 2020 March | 17 | 15 | 32 |
| 2020 February | 3 | 0 | 3 |
| 2019 December | 4 | 0 | 4 |
| 2019 September | 4 | 0 | 4 |
| 2019 June | 2 | 0 | 2 |
| 2019 April | 0 | 5 | 5 |
| 2019 March | 3 | 0 | 3 |
| 2019 February | 2 | 0 | 2 |
| 2019 January | 2 | 0 | 2 |
| 2018 December | 6 | 0 | 6 |
| 2018 November | 3 | 0 | 3 |
| 2018 October | 2 | 0 | 2 |
| 2018 September | 6 | 0 | 6 |
| 2018 February | 34 | 2 | 36 |
| 2018 January | 125 | 17 | 142 |
| 2017 December | 47 | 8 | 55 |
| 2017 November | 32 | 3 | 35 |
| 2017 October | 40 | 8 | 48 |
| 2017 September | 33 | 4 | 37 |
| 2017 August | 36 | 7 | 43 |
| 2017 July | 33 | 8 | 41 |
| 2017 June | 45 | 5 | 50 |
| 2017 May | 23 | 6 | 29 |
| 2017 April | 42 | 13 | 55 |
| 2017 March | 23 | 17 | 40 |
| 2017 February | 27 | 6 | 33 |
| 2017 January | 17 | 8 | 25 |
| 2016 December | 66 | 37 | 103 |
| 2016 November | 20 | 25 | 45 |
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