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the gene that encodes kindlin-1 protein&#44; which is expressed in the skin&#44; periodontal tissue&#44; small intestine&#44; colon&#44; and rectum&#46;<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">5&#44;6</span></a> Kindlin-1 forms part of the cytoskeleton of basal keratinocytes&#44; and participates in cell migration&#44; adhesion&#44; and growth&#46;<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">5&#44;7</span></a> Loss of kindlin-1 function results in skin fragility and defective binding of actin to the extracellular matrix&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">Clinical manifestations usually present from birth and consist of blistering induced by trauma or sun exposure&#44; diffuse skin atrophy&#44; and early-onset poikiloderma in photo-exposed areas&#46; Photosensitivity improves with age&#44; but affected individuals are predisposed to malignant skin tumors in adulthood&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;5</span></a> Mucosal involvement usually begins in adolescence&#59; 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gingival hyperplasia &#40;periodontal pockets and pseudo-pockets&#41;&#59; bacterial plaque&#59; dental calculus&#59; grade 1 mobility in anterior teeth&#59; and dental caries&#46; Whitish&#44; lichenoid-like lesions were observed on the palate &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0045" class="elsevierStylePara elsevierViewall">Immunofluorescence &#40;IF&#41; showed a reticular staining pattern for collagen IV&#44; laminin 332&#44; and collagen VII&#44; and positive antikindlin-1 staining &#40;<a class="elsevierStyleCrossRef" href="#fig0020">Fig&#46; 4</a>&#41;&#46;</p><elsevierMultimedia ident="fig0020"></elsevierMultimedia><p id="par0050" class="elsevierStylePara elsevierViewall">Electron microscopy &#40;EM&#41; showed epidermal segments with normal keratinization and cleavage planes at different levels of the dermoepidermal junction&#44; with the presence of desmosomes and anchoring fibrils &#40;<a class="elsevierStyleCrossRef" href="#fig0025">Fig&#46; 5</a>&#41;&#46;</p><elsevierMultimedia ident="fig0025"></elsevierMultimedia><p id="par0055" class="elsevierStylePara elsevierViewall">Permanent treatment with sunscreen and skin moisturizer was recommended&#46; The patient underwent preventive&#44; palliative&#44; symptomatic&#44; and recuperative dental treatment consisting of scaling and manual and ultrasonic curettage combined with dietary changes and nutritional supplementation for chronic malnutrition&#46;</p><p id="par0060" class="elsevierStylePara elsevierViewall">After 3 months&#44; the patient&#8217;s skin condition and periodontal health improved considerably&#44; and the periodontogram showed marked changes&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Case 2</span><p id="par0065" class="elsevierStylePara elsevierViewall">The patient was the 11-year-old brother of the patient in Case 1&#44; and had skin lesions that were present from birth&#46; In the 2 years preceding the consultation he experienced recurrent episodes of diarrhea alternating with constipation and encopresis&#44; requiring use of a diaper&#46; Physical examination revealed dry skin with atrophic areas&#44; hyperpigmentation and poikiloderma on the face&#44; neck&#44; and chest&#44; and pseudosyndactyly of the hands and feet &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46;</p><p id="par0070" class="elsevierStylePara elsevierViewall">A dental examination revealed dry lips&#44; dyschromia&#44; angular cheilitis&#44; and microstomia&#59; gingival hyperplasia&#44; bacterial plaque&#44; and dental calculus in the upper teeth&#59; and marked loss of bone support in the lower central and lateral incisors with grade 3 mobility&#46; The patient was chronically malnourished and of short stature&#46;</p><p id="par0075" class="elsevierStylePara elsevierViewall">IF revealed a positive reticular staining pattern for collagen IV and VII and laminin 5&#44; and positive antikindlin-1 staining&#46; Blisters at various levels in the dermoepidermal junction were observed on EM&#46; Barium X-ray of the colon showed dolichomegacolon&#46;</p><p id="par0080" class="elsevierStylePara elsevierViewall">Permanent treatment with sunscreen and skin moisturizer was recommended&#44; as well as dental scaling and curettage&#46; The gastroenterology department proposed treatment with osmotic laxatives and dietary changes for the management of colitis and encopresis&#44; as well as nutritional supplementation&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Case 3</span><p id="par0085" class="elsevierStylePara elsevierViewall">The patient was a 15-year-old boy from a small town in the Peruvian Amazon who was born to non-consanguineous parents&#46; He was seen for blistering lesions that formed in response to minimal friction and had first appeared at 2 months of age&#46; Physical examination revealed dyschromia and multiple lentigines in photo-exposed areas and on the labial mucosa&#59; poikiloderma on the face&#44; neck&#44; and the dorsal aspects of the hands and feet&#59; pseudosyndactyly&#59; and plantar pitting &#40;Figs&#46; 2B and 6&#41;&#46; The patient also presented gingival hypertrophy and dental caries&#46;</p><p id="par0090" class="elsevierStylePara elsevierViewall">IF revealed a positive reticular staining pattern for collagen IV and VII and laminin 332&#46; EM showed blistering due to cytolysis in the basal layer&#44; lamina lucida&#44; and lamina densa&#44; and the presence of anchor filaments and fibrils&#46;</p><p id="par0095" class="elsevierStylePara elsevierViewall">Treatment consisted of the use of sunscreen and moisturizers&#44; dental scaling and curettage&#44; and nutritional and psychological evaluation&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Case 4</span><p id="par0100" class="elsevierStylePara elsevierViewall">The patient was a 10-year-old girl&#44; who was the sister of the patient in case 3&#46; From the age of 6 months she presented bullous lesions that left dyschromic scars&#59; skin atrophy&#59; poikiloderma&#59; and freckles predominantly in photo-exposed areas&#46; She had pseudosyndactyly of the hands&#44; gingival hypertrophy&#44; and dental caries&#46; The ophthalmological examination revealed conjunctivitis and mild bilateral ectropion of the lower eyelid &#40;<a class="elsevierStyleCrossRef" href="#fig0030">Fig&#46; 6</a>&#41;&#46;</p><elsevierMultimedia ident="fig0030"></elsevierMultimedia><p id="par0105" class="elsevierStylePara elsevierViewall">IF and EM revealed findings compatible with KS&#46; Management of the patient included the permanent use of artificial tears and lenses with protection against ultraviolet A and B light&#46;</p></span></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Discussion</span><p id="par0110" class="elsevierStylePara elsevierViewall">KS is an autosomal recessive condition&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> While most reported patients are descendants of consanguineous parents &#40;Cases 1 and 2&#41;&#44; KS can also occur as a result of spontaneous mutation&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">10</span></a> While the parents of Cases 3 and 4 denied kinship&#44; they both come from small border towns&#46; If we assume that the parents of the 4 cases described here are asymptomatic heterozygous carriers&#44; the probability of each couple having a child with KS is 25&#37; per pregnancy&#44;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> hence the importance of providing patients and their parents with genetic counseling&#46;</p><p id="par0115" class="elsevierStylePara elsevierViewall">In KS&#44; a mutation in the <span class="elsevierStyleItalic">FERMT1</span> gene results in loss of function&#44; leading to keratinocyte detachment&#44; duplication of the lamina densa&#44; and multiple cleavage planes at different levels of the dermoepidermal junction&#44; beginning at birth&#46;<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">5&#44;7</span></a> From the first years of life all 4 patients presented skin fragility that subsequently diminished&#44; leaving patients with cutaneous atrophy and poikiloderma predominantly in photo-exposed areas&#46; In addition&#44; all 4 patients presented pseudosyndactyly that did not require surgical treatment as it did not compromise functionality&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> All patients presented plantar pitting&#46;</p><p id="par0120" class="elsevierStylePara elsevierViewall">Blistering and photosensitivity improve with age&#44; while poikiloderma and skin atrophy persist&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> Around 10&#37; of patients with KS can develop malignant skin tumors&#44;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;11</span></a> such as squamous cell carcinoma&#44; after 45 years of age&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> The mechanism underlying carcinogenesis may be related to low levels of transforming growth factor &#946; and increased Wnt signaling&#44; both of which have been described in humans and mice with KS&#46;<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">12&#44;13</span></a> The use of sunscreen and protective clothing is essential to minimize the risk of carcinogenesis in these patients&#44; especially in countries with high levels of solar radiation&#44; such as Peru&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#44;13</span></a></p><p id="par0125" class="elsevierStylePara elsevierViewall">Involvement of the mucosa&#44; especially the oral mucosa &#40;85&#37;&#41;&#44; is frequent&#59; patients can present with periodontitis&#44; gingival hyperplasia&#44; premature tooth loss&#44; caries&#44; and halitosis&#44;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">14</span></a> resulting in a vicious cycle of poor hygiene and periodontal disease&#46; All 4 patients presented alterations in the oral mucosa&#44; and received preventive&#44; palliative&#44; and recuperative treatment for chronically affected periodontal tissue&#46; Treatment responses were favorable&#44; but unstable and transitory&#46; These patients should therefore continue to receive supportive periodontal therapy to improve oral hygiene&#46;</p><p id="par0130" class="elsevierStylePara elsevierViewall">Involvement of the ocular&#44; esophageal&#44; anal&#44; and urogenital mucosa is more frequent after 10 years of age&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> The patient in Case 4 presented conjunctivitis and mild ectropion in the lower eyelid&#44; which was managed by the ophthalmology department&#46; Intestinal involvement in the form of colitis due to epitheliolysis &#40;15&#37;&#41;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> is more severe in the complete absence of kindlin-1&#59; intestinal kindlin-1 isoforms can compensate for partial kindlin-1 absence and therefore not all patients have digestive symptoms&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> In Cases 1 and 2&#44; episodes of colitis were reported&#44; associated in Case 2 with encopresis&#44; which led to chronic malnutrition and short stature&#46;</p><p id="par0135" class="elsevierStylePara elsevierViewall">Diagnosis of KS is clinical&#44; supported by IF findings showing an intense&#44; widespread&#44; cross-linked immunostaining pattern in the dermoepidermal membrane for laminin 332 and collagen types IV and VII&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#44;15&#44;16</span></a> Positive immunostaining for antikindlin-1 is also considered diagnostic&#46; Cleavage planes are a characteristic finding of this condition&#44;<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">18</span></a> and are visible on EM in intraepidermal&#44; intralamina lucida&#44; and sublamina densa regions&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#44;17</span></a> All previously described findings were observed in our 4 patients&#46;</p><p id="par0140" class="elsevierStylePara elsevierViewall">Diagnosis can be confirmed using molecular genetic techniques to verify mutation of <span class="elsevierStyleItalic">FERMT</span>&#46;<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">5&#44;13</span></a> That this type of analysis was not feasible in our patients was one of the limitations of the study&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Conclusion</span><p id="par0145" class="elsevierStylePara elsevierViewall">KS is a subtype of EB that is very rarely reported in the medical literature&#46; We present the first 4 cases reported in Peru and describe the specific presentations and associated clinical manifestations&#46; We wish to emphasize the importance of managing these patients using a multidisciplinary approach in order to establish a timely diagnosis and to adequately control clinical signs and prevent complications&#44; thereby improving patient prognosis and quality of life&#46;</p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Conflicts of interest</span><p id="par0150" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest&#46;</p></span></span>"
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          "titulo" => "Introduction"
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              "titulo" => "Case 1"
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              "titulo" => "Case 2"
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              "titulo" => "Case 3"
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    "fechaRecibido" => "2018-12-29"
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            0 => "Kindler syndrome"
            1 => "Epidermolysis bullosa"
            2 => "Poikiloderma"
            3 => "Photosensitivity"
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            0 => "Sindrome de Kindler"
            1 => "Epiderm&#243;lisis bullosa"
            2 => "Poiquilodermia"
            3 => "Fotosensibilidad"
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        "titulo" => "Abstract"
        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Kindler syndrome is a very rare form of bullous epidermolysis&#46; It is a hereditary condition caused by a mutation in the <span class="elsevierStyleItalic">FERMT1</span> gene that encodes the protein kindlin-1&#46; It is clinically characterized by trauma-induced blistering&#44; diffuse skin atrophy&#44; poikiloderma&#44; pseudosyndactylyl&#44; and photosensitivity&#46; The most common mucosal manifestations are conjunctivitis&#44; ectropion&#44; hemorrhagic gingivitis&#44; periodontal disease&#44; premature tooth loss&#44; and severe colitis&#46; We present the first 4 cases of Kindler syndrome diagnosed at the Instituto Nacional de Salud del Ni&#241;o in Lima&#44; Peru&#46; These cases highlight the unique clinical presentation and multiple manifestations of this disease and show how a multidisciplinary management approach kept symptoms under control and significantly improved patient quality of life&#46;</p></span>"
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        "titulo" => "Resumen"
        "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">El S&#237;ndrome de Kindler&#44; es un subtipo de epiderm&#243;lisis bullosa hereditaria muy rara&#44; causada por la mutaci&#243;n del gen FERMT1 que codifica la prote&#237;na kindlina1&#46;</p><p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">Cl&#237;nicamente se caracteriza por la formaci&#243;n de ampollas inducidas por trauma&#44; atrofia cut&#225;nea difusa&#44; poiquilodermia&#44; pseudosindactilia y fotosensibilidad&#46; En mucosas las manifestaciones mas frecuentes incluyen conjuntivitis&#44; ectropi&#243;n&#44; gingivitis hemorr&#225;gicas&#44; enfermedad periodontal&#44; p&#233;rdida prematura de dientes y colitis severa&#46;</p><p id="spar0050" class="elsevierStyleSimplePara elsevierViewall">Presentamos los 4 primeros casos con S&#237;ndrome de Kindler&#44; diagnosticados en el Instituto Nacional de Salud del Ni&#241;o Lima-Per&#250;&#59; con el fin de dar a conocer su particular forma de presentaci&#243;n y variedad de manifestaciones cl&#237;nicas&#44; enfatizando en que este hecho oblig&#243; a realizar un manejo multidisciplinario&#44; que permiti&#243; un control adecuado de los s&#237;ntomas y una notable mejor&#237;a en su calidad de vida&#46;</p></span>"
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        "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as&#58; Torres-Iberico R&#44; Condori-Fern&#225;ndez Y&#44; Apag&#252;e&#241;o-Ruiz C&#44; Andia-Ticona M&#44; Pomar-Morante R&#46; S&#237;ndrome de Kindler&#44; manejo multidisciplinario&#46; Actas Dermosifiliogr&#46; 2020&#46; <span class="elsevierStyleInterRef" id="intr0005" href="https://doi.org/10.1016/j.ad.2019.04.013">https&#58;&#47;&#47;doi&#46;org&#47;10&#46;1016&#47;j&#46;ad&#46;2019&#46;04&#46;013</span></p>"
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          "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Clinical characteristics of Cases 1 &#40;left&#41; and 2 &#40;right&#41;&#46; Dry skin&#44; atrophic areas with hypopigmentation and hyperpigmentation &#40;poikiloderma&#41;&#44; and multiple freckles located predominantly in photo-exposed areas&#46; Both patients presented with pseudosyndactyly of the hands&#46;</p>"
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          "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Clinical manifestations&#46; A&#44; Case 2&#46; Dry skin with atrophic areas with a cigarette-paper-like appearance and multiple freckles&#46; B&#44; Case 3&#46; Plantar pitting&#46;</p>"
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          "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Dental evaluation of Case 1&#46; A&#44; Gingivitis&#44; periodontitis&#44; and gingival hyperplasia &#40;periodontal pockets and pseudo-pockets&#41;&#44; with bacterial plaque&#44; dental calculus&#44; and grade 1 mobility in anterior teeth&#46; B&#44; Post-treatment view of teeth in occlusion in which a marked clinical improvement is evident&#46; C&#44; Panoramic radiograph showing horizontal resorption of interdental crests&#46;</p>"
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          "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Case 1&#46; Immunofluorescence showing a positive cross-linked staining pattern in the dermoepidermal membrane for collagen IV &#40;A&#41;&#44; collagen VII &#40;B&#41;&#44; and laminin 332 &#40;C&#41;&#46; This pattern is characteristic of Kindler syndrome&#46;</p>"
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          "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Case 1&#46; Electron microscopy&#46; Serial ultrafine sections &#40;100-nm thick&#41; showing blisters &#40;arrows&#41; at different levels of the dermo-epidermal junction&#44; with desmosomes and anchoring fibrils without alterations&#46;</p>"
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          "en" => "<p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Clinical manifestations in Cases 3 and 4&#46; Both patients presented with skin atrophy&#44; hypochromic and hyperchromic areas with multiple telangiectasias &#40;poikiloderma&#41;&#44; and freckles predominantly in photo-exposed areas&#44; as well as conjunctivitis and mild bilateral ectropion of the lower eyelid &#40;right-hand image&#41;&#46;</p>"
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    "bibliografia" => array:2 [
      "titulo" => "References"
      "seccion" => array:1 [
        0 => array:2 [
          "identificador" => "bibs0005"
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              "etiqueta" => "1"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Congenital poikiloderma with traumatic bulla formation and progressive cutaneous atrophy"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:1 [
                            0 => "T&#46; Kindler"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1111/j.1365-2133.1954.tb12598.x"
                      "Revista" => array:6 [
                        "tituloSerie" => "Br J Dermatol&#46;"
                        "fecha" => "1954"
                        "volumen" => "66"
                        "paginaInicial" => "104"
                        "paginaFinal" => "111"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/13149722"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            1 => array:3 [
              "identificador" => "bib0010"
              "etiqueta" => "2"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "The classification of inherited epidermolysis bullosa &#40;EB&#41;&#58; Report of the Third International Consensus Meeting on Diagnosis and Classification of EB"
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        "texto" => "<p id="par0155" class="elsevierStylePara elsevierViewall">The authors thank Drs&#46; Roxana Lipa Chancolla&#44; Luis Claudio Huaman&#237; Huayhua&#44; Gilmer Torres Ramos&#44; and Rosario Loaiza de la Cruz&#46;</p>"
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Brief Comunication
Kindler Syndrome: A Multidisciplinary Management Approach
Síndrome de Kindler, manejo multidisciplinario
R. Torres-Ibericoa,
Autor para correspondencia
rtorresi@insn.gob.pe

Corresponding author.
, Y. Condori-Fernándezb, C. Apagüeño-Ruiza, M. Andia-Ticonac, R. Pomar-Morantea
a Servicio de Dermatología, Instituto Nacional de Salud del Niño, Breña, Lima, Peru
b Servicio de Dermatología, Hospital Vitarte MINSA, Lima, Peru
c Servicio de Odontología, Instituto Nacional de Salud del Niño, Breña, Lima, Peru
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the gene that encodes kindlin-1 protein&#44; which is expressed in the skin&#44; periodontal tissue&#44; small intestine&#44; colon&#44; and rectum&#46;<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">5&#44;6</span></a> Kindlin-1 forms part of the cytoskeleton of basal keratinocytes&#44; and participates in cell migration&#44; adhesion&#44; and growth&#46;<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">5&#44;7</span></a> Loss of kindlin-1 function results in skin fragility and defective binding of actin to the extracellular matrix&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">Clinical manifestations usually present from birth and consist of blistering induced by trauma or sun exposure&#44; diffuse skin atrophy&#44; and early-onset poikiloderma in photo-exposed areas&#46; Photosensitivity improves with age&#44; but affected individuals are predisposed to malignant skin tumors in adulthood&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;5</span></a> Mucosal involvement usually begins in adolescence&#59; 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5</a>&#41;&#46;</p><elsevierMultimedia ident="fig0025"></elsevierMultimedia><p id="par0055" class="elsevierStylePara elsevierViewall">Permanent treatment with sunscreen and skin moisturizer was recommended&#46; The patient underwent preventive&#44; palliative&#44; symptomatic&#44; and recuperative dental treatment consisting of scaling and manual and ultrasonic curettage combined with dietary changes and nutritional supplementation for chronic malnutrition&#46;</p><p id="par0060" class="elsevierStylePara elsevierViewall">After 3 months&#44; the patient&#8217;s skin condition and periodontal health improved considerably&#44; and the periodontogram showed marked changes&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Case 2</span><p id="par0065" class="elsevierStylePara elsevierViewall">The patient was the 11-year-old brother of the patient in Case 1&#44; and had skin lesions that were present from birth&#46; In the 2 years preceding the consultation he experienced recurrent episodes of diarrhea alternating with constipation and encopresis&#44; requiring use of a diaper&#46; Physical examination revealed dry skin with atrophic areas&#44; hyperpigmentation and poikiloderma on the face&#44; neck&#44; and chest&#44; and pseudosyndactyly of the hands and feet &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46;</p><p id="par0070" class="elsevierStylePara elsevierViewall">A dental examination revealed dry lips&#44; dyschromia&#44; angular cheilitis&#44; and microstomia&#59; gingival hyperplasia&#44; bacterial plaque&#44; and dental calculus in the upper teeth&#59; and marked loss of bone support in the lower central and lateral incisors with grade 3 mobility&#46; The patient was chronically malnourished and of short stature&#46;</p><p id="par0075" class="elsevierStylePara elsevierViewall">IF revealed a positive reticular staining pattern for collagen IV and VII and laminin 5&#44; and positive antikindlin-1 staining&#46; Blisters at various levels in the dermoepidermal junction were observed on EM&#46; Barium X-ray of the colon showed dolichomegacolon&#46;</p><p id="par0080" class="elsevierStylePara elsevierViewall">Permanent treatment with sunscreen and skin moisturizer was recommended&#44; as well as dental scaling and curettage&#46; The gastroenterology department proposed treatment with osmotic laxatives and dietary changes for the management of colitis and encopresis&#44; as well as nutritional supplementation&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Case 3</span><p id="par0085" class="elsevierStylePara elsevierViewall">The patient was a 15-year-old boy from a small town in the Peruvian Amazon who was born to non-consanguineous parents&#46; He was seen for blistering lesions that formed in response to minimal friction and had first appeared at 2 months of age&#46; Physical examination revealed dyschromia and multiple lentigines in photo-exposed areas and on the labial mucosa&#59; poikiloderma on the face&#44; neck&#44; and the dorsal aspects of the hands and feet&#59; pseudosyndactyly&#59; and plantar pitting &#40;Figs&#46; 2B and 6&#41;&#46; The patient also presented gingival hypertrophy and dental caries&#46;</p><p id="par0090" class="elsevierStylePara elsevierViewall">IF revealed a positive reticular staining pattern for collagen IV and VII and laminin 332&#46; EM showed blistering due to cytolysis in the basal layer&#44; lamina lucida&#44; and lamina densa&#44; and the presence of anchor filaments and fibrils&#46;</p><p id="par0095" class="elsevierStylePara elsevierViewall">Treatment consisted of the use of sunscreen and moisturizers&#44; dental scaling and curettage&#44; and nutritional and psychological evaluation&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Case 4</span><p id="par0100" class="elsevierStylePara elsevierViewall">The patient was a 10-year-old girl&#44; who was the sister of the patient in case 3&#46; From the age of 6 months she presented bullous lesions that left dyschromic scars&#59; skin atrophy&#59; poikiloderma&#59; and freckles predominantly in photo-exposed areas&#46; She had pseudosyndactyly of the hands&#44; gingival hypertrophy&#44; and dental caries&#46; The ophthalmological examination revealed conjunctivitis and mild bilateral ectropion of the lower eyelid &#40;<a class="elsevierStyleCrossRef" href="#fig0030">Fig&#46; 6</a>&#41;&#46;</p><elsevierMultimedia ident="fig0030"></elsevierMultimedia><p id="par0105" class="elsevierStylePara elsevierViewall">IF and EM revealed findings compatible with KS&#46; Management of the patient included the permanent use of artificial tears and lenses with protection against ultraviolet A and B light&#46;</p></span></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Discussion</span><p id="par0110" class="elsevierStylePara elsevierViewall">KS is an autosomal recessive condition&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> While most reported patients are descendants of consanguineous parents &#40;Cases 1 and 2&#41;&#44; KS can also occur as a result of spontaneous mutation&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">10</span></a> While the parents of Cases 3 and 4 denied kinship&#44; they both come from small border towns&#46; If we assume that the parents of the 4 cases described here are asymptomatic heterozygous carriers&#44; the probability of each couple having a child with KS is 25&#37; per pregnancy&#44;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> hence the importance of providing patients and their parents with genetic counseling&#46;</p><p id="par0115" class="elsevierStylePara elsevierViewall">In KS&#44; a mutation in the <span class="elsevierStyleItalic">FERMT1</span> gene results in loss of function&#44; leading to keratinocyte detachment&#44; duplication of the lamina densa&#44; and multiple cleavage planes at different levels of the dermoepidermal junction&#44; beginning at birth&#46;<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">5&#44;7</span></a> From the first years of life all 4 patients presented skin fragility that subsequently diminished&#44; leaving patients with cutaneous atrophy and poikiloderma predominantly in photo-exposed areas&#46; In addition&#44; all 4 patients presented pseudosyndactyly that did not require surgical treatment as it did not compromise functionality&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> All patients presented plantar pitting&#46;</p><p id="par0120" class="elsevierStylePara elsevierViewall">Blistering and photosensitivity improve with age&#44; while poikiloderma and skin atrophy persist&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> Around 10&#37; of patients with KS can develop malignant skin tumors&#44;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;11</span></a> such as squamous cell carcinoma&#44; after 45 years of age&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> The mechanism underlying carcinogenesis may be related to low levels of transforming growth factor &#946; and increased Wnt signaling&#44; both of which have been described in humans and mice with KS&#46;<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">12&#44;13</span></a> The use of sunscreen and protective clothing is essential to minimize the risk of carcinogenesis in these patients&#44; especially in countries with high levels of solar radiation&#44; such as Peru&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#44;13</span></a></p><p id="par0125" class="elsevierStylePara elsevierViewall">Involvement of the mucosa&#44; especially the oral mucosa &#40;85&#37;&#41;&#44; is frequent&#59; patients can present with periodontitis&#44; gingival hyperplasia&#44; premature tooth loss&#44; caries&#44; and halitosis&#44;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">14</span></a> resulting in a vicious cycle of poor hygiene and periodontal disease&#46; All 4 patients presented alterations in the oral mucosa&#44; and received preventive&#44; palliative&#44; and recuperative treatment for chronically affected periodontal tissue&#46; Treatment responses were favorable&#44; but unstable and transitory&#46; These patients should therefore continue to receive supportive periodontal therapy to improve oral hygiene&#46;</p><p id="par0130" class="elsevierStylePara elsevierViewall">Involvement of the ocular&#44; esophageal&#44; anal&#44; and urogenital mucosa is more frequent after 10 years of age&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> The patient in Case 4 presented conjunctivitis and mild ectropion in the lower eyelid&#44; which was managed by the ophthalmology department&#46; Intestinal involvement in the form of colitis due to epitheliolysis &#40;15&#37;&#41;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> is more severe in the complete absence of kindlin-1&#59; intestinal kindlin-1 isoforms can compensate for partial kindlin-1 absence and therefore not all patients have digestive symptoms&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> In Cases 1 and 2&#44; episodes of colitis were reported&#44; associated in Case 2 with encopresis&#44; which led to chronic malnutrition and short stature&#46;</p><p id="par0135" class="elsevierStylePara elsevierViewall">Diagnosis of KS is clinical&#44; supported by IF findings showing an intense&#44; widespread&#44; cross-linked immunostaining pattern in the dermoepidermal membrane for laminin 332 and collagen types IV and VII&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#44;15&#44;16</span></a> Positive immunostaining for antikindlin-1 is also considered diagnostic&#46; Cleavage planes are a characteristic finding of this condition&#44;<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">18</span></a> and are visible on EM in intraepidermal&#44; intralamina lucida&#44; and sublamina densa regions&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#44;17</span></a> All previously described findings were observed in our 4 patients&#46;</p><p id="par0140" class="elsevierStylePara elsevierViewall">Diagnosis can be confirmed using molecular genetic techniques to verify mutation of <span class="elsevierStyleItalic">FERMT</span>&#46;<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">5&#44;13</span></a> That this type of analysis was not feasible in our patients was one of the limitations of the study&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Conclusion</span><p id="par0145" class="elsevierStylePara elsevierViewall">KS is a subtype of EB that is very rarely reported in the medical literature&#46; We present the first 4 cases reported in Peru and describe the specific presentations and associated clinical manifestations&#46; We wish to emphasize the importance of managing these patients using a multidisciplinary approach in order to establish a timely diagnosis and to adequately control clinical signs and prevent complications&#44; thereby improving patient prognosis and quality of life&#46;</p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Conflicts of interest</span><p id="par0150" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest&#46;</p></span></span>"
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          "titulo" => "Introduction"
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            0 => array:2 [
              "identificador" => "sec0010"
              "titulo" => "Case 1"
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              "titulo" => "Case 2"
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              "identificador" => "sec0020"
              "titulo" => "Case 3"
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              "titulo" => "Case 4"
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    "fechaRecibido" => "2018-12-29"
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            0 => "Kindler syndrome"
            1 => "Epidermolysis bullosa"
            2 => "Poikiloderma"
            3 => "Photosensitivity"
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            0 => "Sindrome de Kindler"
            1 => "Epiderm&#243;lisis bullosa"
            2 => "Poiquilodermia"
            3 => "Fotosensibilidad"
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        "titulo" => "Abstract"
        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Kindler syndrome is a very rare form of bullous epidermolysis&#46; It is a hereditary condition caused by a mutation in the <span class="elsevierStyleItalic">FERMT1</span> gene that encodes the protein kindlin-1&#46; It is clinically characterized by trauma-induced blistering&#44; diffuse skin atrophy&#44; poikiloderma&#44; pseudosyndactylyl&#44; and photosensitivity&#46; The most common mucosal manifestations are conjunctivitis&#44; ectropion&#44; hemorrhagic gingivitis&#44; periodontal disease&#44; premature tooth loss&#44; and severe colitis&#46; We present the first 4 cases of Kindler syndrome diagnosed at the Instituto Nacional de Salud del Ni&#241;o in Lima&#44; Peru&#46; These cases highlight the unique clinical presentation and multiple manifestations of this disease and show how a multidisciplinary management approach kept symptoms under control and significantly improved patient quality of life&#46;</p></span>"
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        "titulo" => "Resumen"
        "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">El S&#237;ndrome de Kindler&#44; es un subtipo de epiderm&#243;lisis bullosa hereditaria muy rara&#44; causada por la mutaci&#243;n del gen FERMT1 que codifica la prote&#237;na kindlina1&#46;</p><p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">Cl&#237;nicamente se caracteriza por la formaci&#243;n de ampollas inducidas por trauma&#44; atrofia cut&#225;nea difusa&#44; poiquilodermia&#44; pseudosindactilia y fotosensibilidad&#46; En mucosas las manifestaciones mas frecuentes incluyen conjuntivitis&#44; ectropi&#243;n&#44; gingivitis hemorr&#225;gicas&#44; enfermedad periodontal&#44; p&#233;rdida prematura de dientes y colitis severa&#46;</p><p id="spar0050" class="elsevierStyleSimplePara elsevierViewall">Presentamos los 4 primeros casos con S&#237;ndrome de Kindler&#44; diagnosticados en el Instituto Nacional de Salud del Ni&#241;o Lima-Per&#250;&#59; con el fin de dar a conocer su particular forma de presentaci&#243;n y variedad de manifestaciones cl&#237;nicas&#44; enfatizando en que este hecho oblig&#243; a realizar un manejo multidisciplinario&#44; que permiti&#243; un control adecuado de los s&#237;ntomas y una notable mejor&#237;a en su calidad de vida&#46;</p></span>"
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        "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as&#58; Torres-Iberico R&#44; Condori-Fern&#225;ndez Y&#44; Apag&#252;e&#241;o-Ruiz C&#44; Andia-Ticona M&#44; Pomar-Morante R&#46; S&#237;ndrome de Kindler&#44; manejo multidisciplinario&#46; Actas Dermosifiliogr&#46; 2020&#46; <span class="elsevierStyleInterRef" id="intr0005" href="https://doi.org/10.1016/j.ad.2019.04.013">https&#58;&#47;&#47;doi&#46;org&#47;10&#46;1016&#47;j&#46;ad&#46;2019&#46;04&#46;013</span></p>"
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          "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Clinical characteristics of Cases 1 &#40;left&#41; and 2 &#40;right&#41;&#46; Dry skin&#44; atrophic areas with hypopigmentation and hyperpigmentation &#40;poikiloderma&#41;&#44; and multiple freckles located predominantly in photo-exposed areas&#46; Both patients presented with pseudosyndactyly of the hands&#46;</p>"
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          "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Clinical manifestations&#46; A&#44; Case 2&#46; Dry skin with atrophic areas with a cigarette-paper-like appearance and multiple freckles&#46; B&#44; Case 3&#46; Plantar pitting&#46;</p>"
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          "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Dental evaluation of Case 1&#46; A&#44; Gingivitis&#44; periodontitis&#44; and gingival hyperplasia &#40;periodontal pockets and pseudo-pockets&#41;&#44; with bacterial plaque&#44; dental calculus&#44; and grade 1 mobility in anterior teeth&#46; B&#44; Post-treatment view of teeth in occlusion in which a marked clinical improvement is evident&#46; C&#44; Panoramic radiograph showing horizontal resorption of interdental crests&#46;</p>"
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          "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Case 1&#46; Immunofluorescence showing a positive cross-linked staining pattern in the dermoepidermal membrane for collagen IV &#40;A&#41;&#44; collagen VII &#40;B&#41;&#44; and laminin 332 &#40;C&#41;&#46; This pattern is characteristic of Kindler syndrome&#46;</p>"
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          "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Case 1&#46; Electron microscopy&#46; Serial ultrafine sections &#40;100-nm thick&#41; showing blisters &#40;arrows&#41; at different levels of the dermo-epidermal junction&#44; with desmosomes and anchoring fibrils without alterations&#46;</p>"
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          "en" => "<p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Clinical manifestations in Cases 3 and 4&#46; Both patients presented with skin atrophy&#44; hypochromic and hyperchromic areas with multiple telangiectasias &#40;poikiloderma&#41;&#44; and freckles predominantly in photo-exposed areas&#44; as well as conjunctivitis and mild bilateral ectropion of the lower eyelid &#40;right-hand image&#41;&#46;</p>"
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    "bibliografia" => array:2 [
      "titulo" => "References"
      "seccion" => array:1 [
        0 => array:2 [
          "identificador" => "bibs0005"
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              "etiqueta" => "1"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Congenital poikiloderma with traumatic bulla formation and progressive cutaneous atrophy"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:1 [
                            0 => "T&#46; Kindler"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1111/j.1365-2133.1954.tb12598.x"
                      "Revista" => array:6 [
                        "tituloSerie" => "Br J Dermatol&#46;"
                        "fecha" => "1954"
                        "volumen" => "66"
                        "paginaInicial" => "104"
                        "paginaFinal" => "111"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/13149722"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            1 => array:3 [
              "identificador" => "bib0010"
              "etiqueta" => "2"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "The classification of inherited epidermolysis bullosa &#40;EB&#41;&#58; Report of the Third International Consensus Meeting on Diagnosis and Classification of EB"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
                            0 => "J&#46;-D&#46; Fine"
                            1 => "R&#46;A&#46;J&#46; Eady"
                            2 => "E&#46;A&#46; Bauer"
                            3 => "J&#46;W&#46; Bauer"
                            4 => "L&#46; Bruckner-Tuderman"
                            5 => "A&#46; Heagerty"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:5 [
                        "tituloSerie" => "J Am Acad Dermatol&#46;"
                        "fecha" => "2008"
                        "volumen" => "58"
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                    ]
                  ]
                ]
              ]
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