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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Case presentation</span><p id="par0005" class="elsevierStylePara elsevierViewall">We report the case of a previously healthy 51-year-old male patient that presented with a twelve-month history of skin lesions located at the trunk&#46; The lesions caused local burning and were exacerbated by heat and sweating&#46; There were no associated cutaneous or extracutaneous symptoms and the patient denied any relevant family history&#46; On physical examination&#44; there were multiple grouped erythematous keratotic papules and erosions in a blaschkoid distribution on the trunk &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; The patient had no mucosal or nail lesions&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">Lesional biopsy revealed multiple foci of acantholysis and dyskeratosis&#44; grains and occasional corps ronds &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">What is the diagnosis&#63;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Diagnosis</span><p id="par0020" class="elsevierStylePara elsevierViewall">Segmental Darier Disease &#40;type 1&#41;&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Comments</span><p id="par0025" class="elsevierStylePara elsevierViewall">Darier disease is an autosomal dominant genodermatosis&#44; characterized by the presence of mutations in the <span class="elsevierStyleItalic">ATP2A2</span> gene&#46; These mutations result in dysfunction of an endoplasmic reticulum Ca<span class="elsevierStyleSup">2&#43;</span> ATPase &#40;SERCA2&#41;&#44; thus interfering in the signaling of intracellular calcium&#46; Consequently&#44; there is loss of supra-basal cell adhesion &#40;acantholysis&#41; and induction of apoptosis &#40;dyskeratosis&#41;&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">From a clinical point of view&#44; classic Darier disease is characterized by the presence of crusted greasy keratotic papules and plaques&#44; located predominantly in seborrheic areas&#46; Additional findings include palmoplantar papules&#44; whitish papules on the oral mucosa and variable nail changes&#44; including V-shaped notching and alternating white and red longitudinal streaks&#44; which is considered pathognomonic&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">1</span></a> Environmental factors&#44; including heat&#44; friction&#44; sweating and UV light can induce flares&#44; and some medications are also known to cause exacerbations&#44; namely lithium&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">2</span></a> Darier disease follows a chronic course with oscillations in disease severity and secondary bacterial and viral infections are frequent complications&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">In addition to the more classic presentation&#44; two types of segmental Darier disease have been described&#44; both with lesions distributed along the lines of Blaschko&#46; The most common subtype is type 1&#44; which is the result of a post-zygotic mutation in the <span class="elsevierStyleItalic">ATP2A2</span> gene during embryogenesis&#44; thus prompting cutaneous mosaic involvement&#46; In type 1 segmental disease&#44; the age of onset&#44; severity and histological findings are similar to the generalized Darier form&#46; Type 2 mosaicism is more severe and less frequent&#46; These patients present with generalized Darier disease as well as additional linear lesions&#44; with increased severity&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">3</span></a> Type 2 segmental disease occurs when patients carrying a heterozygous germline mutation have an additional postzygotic inactivating mutation in the other allele of the same gene&#44; causing loss of heterozygosity&#46; The histological findings described in this patient evoke the diagnosis of Darier&#39;s Disease&#59; in light of the clinical picture described&#44; this case corresponds to type 1 segmental disease&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">The therapeutic approach to Darier disease is often difficult and challenging&#46; General measures including avoidance of heat and minimization of friction are helpful&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">2</span></a> In addition&#44; corticosteroids can be used to reduce inflammation and topical antimicrobial washes may help decrease the risk of superimposed infection&#46; Systemic retinoids and low-dose naltrexone have been used in some cases with good results&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">2</span></a> Surgical approach is an option in severe refractory cases&#46; Our patient was started on acitretin 10<span class="elsevierStyleHsp" style=""></span>mg daily with marked improvement and remains under surveillance&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">The clinical differential diagnosis of type 1 segmental Darier disease includes Blaschkoid linear acquired inflammatory skin eruption&#44; linear lichen planus&#44; linear porokeratosis and nevus unis lateris&#46; Histopathology findings are of major importance&#44; establishing the diagnosis&#46; Early diagnosis will help avoid unnecessary and costly treatment for unrelated conditions&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Conflict of interests</span><p id="par0050" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflict of interest&#46;</p></span></span>"
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