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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Hailey-Hailey disease is a genodermatosis of autosomal dominant transmission&#44; caused by loss-of-function mutations in the ATP2C1 gene&#46; Failure of a proper intracellular calcium homeostasis&#44; particularly in the Golgi apparatus&#44; is responsible for a weakened desmosomal adhesion between keratinocytes&#46; Thus&#44; acantholysis of stratum spinosum occurs&#44; causing a chronic clinical appearance of flaccid blisters and malodourous erosions in intertriginous areas&#46; Chronic pruritus is also frequently present and difficult to treat&#44; contributing to the severe burden of the disease&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">A 57-year-old woman suffered from Hailey-Hailey disease since early adulthood&#44; which was refractory to several different topical and systemic treatments&#44; including a recently described novel approach with low-dose naltrexone&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">1</span></a> She had malodourous bilateral hyperkeratotic papules and erosions on axillae&#44; inframammary and abdominal folds &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>a&#41;&#46; Pruritus was her main complaint&#44; as it was persistent&#44; intense &#40;Numeric Rating Scale itch 8&#47;10&#41; and disruptive of both her sleep quality and social life&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Dupilumab 300<span class="elsevierStyleHsp" style=""></span>mg every other week was started after a 600<span class="elsevierStyleHsp" style=""></span>mg loading dose&#46; Both pruritus and sleep quality drastically improved after only one week of treatment&#46; Additionally&#44; significant healing of the skin lesions was noticeable by week 12&#44; with reduction in size and thickness of previous hyperkeratotic papules and erosions &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>b&#41;&#46; Treatment was well tolerated&#44; and the patient is satisfied with the outcome&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">Our case reflects a remarkable improvement with dupilumab&#44; a fully human monoclonal antibody against interleukins &#40;IL&#41; 4 and 13&#44; on both subjective and objective findings in Hailey-Hailey disease&#44; thus corroborating the findings by Alzahrani et al&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">2</span></a> This drug is currently approved for moderate-to-severe atopic dermatitis&#44; showing significant reduction of pruritus right after the induction phase&#46; Even though the mechanism of dupilumab on Hailey-Hailey disease is still unknown&#44; it is possible that the inhibition of IL-4 and IL-13 modulates favourably the intracellular calcium signalling&#44;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">2</span></a> thus compensating its genetic defect&#46; Furthermore&#44; we emphasize the central role of IL-4 as a neuromodulator on chronic pruritus&#44; which is responsible for amplifying responses to minor pruritogenic agents&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">3&#44;4</span></a> The expression of IL-4 receptors on sensory neurons of the dorsal root ganglion<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">4</span></a> further corroborates the relevance of this molecule in chronic pruritus&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">In summary&#44; we highlight dupilumab&#39;s innovative role on managing symptoms and objective findings in patients with Hailey-Hailey disease&#46; It is possible that this positive effect could be extrapolated to other dyskeratosis with severe pruritus&#44; but further investigation is needed&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflicts of interest</span><p id="par0030" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest&#46;</p></span></span>"
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