{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,1]],"date-time":"2026-02-01T12:42:03Z","timestamp":1769949723730,"version":"3.49.0"},"reference-count":29,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2014,11,28]],"date-time":"2014-11-28T00:00:00Z","timestamp":1417132800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Int J Dermatology"],"published-print":{"date-parts":[[2015,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Hailey\u2013Hailey disease (<jats:styled-content style=\"fixed-case\">HHD<\/jats:styled-content>), also known as familial benign chronic pemphigus, is a rare autosomal dominant inherited intraepidermal blistering genodermatosis. Mutations in the <jats:styled-content style=\"fixed-case\">ATP<\/jats:styled-content>2<jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>1 gene encoding for the <jats:styled-content style=\"fixed-case\">G<\/jats:styled-content>olgi secretory pathway <jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>a<jats:sup>2+<\/jats:sup>\/<jats:styled-content style=\"fixed-case\">M<\/jats:styled-content>n<jats:sup>2+<\/jats:sup>\u2010<jats:styled-content style=\"fixed-case\">ATP<\/jats:styled-content>asi protein 1 (<jats:styled-content style=\"fixed-case\">SPCA<\/jats:styled-content>1) affect the processing of desmosomal components and the epidermal suprabasal cell\u2013cell adhesion by deregulating the keratinocyte cytosolic <jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>a<jats:sup>2+<\/jats:sup> concentration. We report the unexpected, dramatic, and persistent clinical improvement of the skin lesions of a patient affected with longstanding <jats:styled-content style=\"fixed-case\">HHD<\/jats:styled-content> with daily intake of a solution containing magnesium chloride hexahydrate (<jats:styled-content style=\"fixed-case\">M<\/jats:styled-content>g<jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>l<jats:sub>2<\/jats:sub>).<\/jats:p><\/jats:sec><jats:sec><jats:title>Materials and methods<\/jats:title><jats:p>We investigated the effect of <jats:styled-content style=\"fixed-case\">M<\/jats:styled-content>g<jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>l<jats:sub>2<\/jats:sub> on the intracellular <jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>a<jats:sup>2+<\/jats:sup> homeostasis and on the activity of particular <jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>a<jats:sup>2+<\/jats:sup>\u2010effectors in <jats:styled-content style=\"fixed-case\">H<\/jats:styled-content>e<jats:styled-content style=\"fixed-case\">L<\/jats:styled-content>a cells transfected with chimeric aequorins (cyt<jats:styled-content style=\"fixed-case\">AEQ<\/jats:styled-content>, mt<jats:styled-content style=\"fixed-case\">AEQ<\/jats:styled-content>, er<jats:styled-content style=\"fixed-case\">AEQ<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">G<\/jats:styled-content>o<jats:styled-content style=\"fixed-case\">AEQ<\/jats:styled-content>) targeted to different subcellular compartments (cytosol, mitochondria, endoplasmic reticulum, and <jats:styled-content style=\"fixed-case\">G<\/jats:styled-content>olgi, respectively).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Experimental investigations on <jats:styled-content style=\"fixed-case\">H<\/jats:styled-content>e<jats:styled-content style=\"fixed-case\">L<\/jats:styled-content>a cells showed the effect of <jats:styled-content style=\"fixed-case\">M<\/jats:styled-content>g<jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>l<jats:sub>2<\/jats:sub> on the function of <jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>a<jats:sup>2+<\/jats:sup>\u2010extrusor systems, resulting in increased cytosolic and mitochondrial <jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>a<jats:sup>2+<\/jats:sup> levels, without altering the mechanisms of intraluminal <jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>a<jats:sup>2+<\/jats:sup>\u2010filling and <jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>a<jats:sup>2+<\/jats:sup>\u2010release of stores.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Based on our clinical observation and experimental results, it can be hypothesized that <jats:styled-content style=\"fixed-case\">M<\/jats:styled-content>g<jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>l<jats:sub>2<\/jats:sub> could act as an inhibitor of the <jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>a<jats:sup>2+<\/jats:sup>\u2010extruding activity in keratinocytes favoring intracellular <jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>a<jats:sup>2+<\/jats:sup>\u2010disponibility and <jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>a<jats:sup>2+<\/jats:sup>\u2010dependent mechanisms in desmosome assembly. This may represent the molecular basis of the good response of the <jats:styled-content style=\"fixed-case\">HHD<\/jats:styled-content> clinical features with <jats:styled-content style=\"fixed-case\">M<\/jats:styled-content>g<jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>l<jats:sub>2<\/jats:sub> solution in the patient described.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ijd.12410","type":"journal-article","created":{"date-parts":[[2014,11,28]],"date-time":"2014-11-28T16:00:53Z","timestamp":1417190453000},"page":"543-548","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":20,"title":["Efficacy of magnesium chloride in the treatment of <scp>H<\/scp>ailey\u2013<scp>H<\/scp>ailey disease: from serendipity to evidence of its effect on intracellular <scp>C<\/scp>a<sup>2+<\/sup> 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