Letter - Therapy
Topical aluminum chloride as a treatment option for Hailey-Hailey disease: a remarkable therapeutic outcome case report
Maraya de Jesus Semblano Bittencourt
, Pedro Carneiro Marinho, Thereza Christina Frade, Gabriela Athayde Amin, Lorena Silva de Carvalho, Lívia Eloi Castro Santos
Corresponding author
Department of Dermatology, Centro Universitário do Estado do Pará, Belém, PA, Brazil
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Decrease in ulcerous-crusted plaques and blisters, and predominance of residual hyperchromia at the end of treatment.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Hailey-Hailey disease (HHD) or benign familial pemphigus, is a rare autosomal dominant genodermatosis, characterized by chronic and painful blisters and ulcerations in intertriginous areas, which cause significant impairment of quality of life of affected patients.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,2</span></a> This case report describes a 55-year-old woman who had developed painful, recurrent lesions since the age of 13, which started as blisters in the intertriginous areas. Over the years, the patient remained undiagnosed and underwent treatments with oral and topical corticosteroids, topical and systemic antifungals, oral and topical antibiotics, and topical immunomodulators, with slight improvement and frequent relapses, which resulted in significant impairment of her quality of life.</p><p id="par0010" class="elsevierStylePara elsevierViewall">On dermatological examination, multiple ulcerous-crusted plaques were observed, some with intact blisters, disseminated in intertriginous areas of the neck, dorsum, abdomen, groin and axillae (<a class="elsevierStyleCrossRef" href="#fig0005">Figs. 1</a>A, <a class="elsevierStyleCrossRef" href="#fig0010">2</a>A and <a class="elsevierStyleCrossRef" href="#fig0015">3</a>A). Histopathological examination showed a “dilapidated brick wall” appearance of the epidermis, compatible with HHD (<a class="elsevierStyleCrossRef" href="#fig0020">Fig. 4</a>). The patient was advised to use, exclusively on the lesion areas, 15% aluminum chloride in aqueous solution twice a week (Perspirex® roll-on), with almost complete remission after eight weeks, as well as substantial improvement in quality of life (<a class="elsevierStyleCrossRef" href="#fig0005">Figs. 1</a>B, <a class="elsevierStyleCrossRef" href="#fig0010">2</a>B and <a class="elsevierStyleCrossRef" href="#fig0015">3</a>B). The patient is being monitored, with isolated and minor recurrences.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><elsevierMultimedia ident="fig0020"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">HHD lesions usually appear soon after puberty, compromising patients active life years.<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3,4</span></a> Patients quality of life is significantly impaired due to the symptoms (pruritus, burning sensation and pain), foul body odor and chronicity.<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2,4</span></a> Mutations in the ATP2C1 gene, which encodes a Ca<span class="elsevierStyleSup">2+</span> ATPase pump, lead to changes in Ca<span class="elsevierStyleSup">2+</span>-dependent intracellular signaling, resulting in loss of cell adhesion in the epidermis.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">Several topical and systemic treatments are proposed in the literature,<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> such as laser ablation, botulinum toxin type A,<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4,5</span></a> dermabrasion, narrow-band UVB phototherapy, topical 5-fluorouracil, dupilumab,<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> TNF-alpha inhibitors, as well as oral treatment with oxybutynin, apremilast, vitamin D, glycopyrrolate, afamelanotide, dapsone, acitretin and cyclosporine.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,3</span></a> Some of these treatment options are high-cost, others have important side effects and others are not very accessible for most patients.</p><p id="par0025" class="elsevierStylePara elsevierViewall">Sweating is known to worsen and may precipitate the appearance of blisters in patients with HHD, which is why treatments that block sweat production, such as botulinum toxin,<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4,5</span></a> oral oxybutynin, and oral glycopyrrolate have been used with good response in some cases.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> For this reason, treatment with a topical 15% aqueous aluminum chloride solution was chosen. This medication acts at the level of the eccrine gland duct, blocking it and producing atrophy and vacuolation of the secretory glandular cells. An “ionic” effect of the used treatment is also possible, by providing another element (aluminum) in contact with the skin, where intracellular traffic of Ca<span class="elsevierStyleSup">2+</span> and Mg<span class="elsevierStyleSup">2+</span> ions is altered by the defect caused by the disease.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">The patient in this report showed an excellent clinical response, with no local side effects and a considerable reduction in the frequency and intensity of crises. The authors consider this treatment option to be low-cost and effective in reducing the frequency of crises and symptoms of this disabling condition.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Financial support</span><p id="par0035" class="elsevierStylePara elsevierViewall">None declared.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Authors' contributions</span><p id="par0040" class="elsevierStylePara elsevierViewall">Maraya de Jesus Semblano Bittencourt: Collection, analysis and interpretation of data; drafting and editing of the manuscript; effective participation in research orientation; intellectual participation in the propaedeutic conduct of the studied case; approval of the final version of the manuscript.</p><p id="par0045" class="elsevierStylePara elsevierViewall">Pedro Carneiro Marinho: Collection, analysis and interpretation of data; critical review of the literature; drafting and editing of the manuscript; intellectual participation in the propaedeutic conduct of the studied case.</p><p id="par0050" class="elsevierStylePara elsevierViewall">Thereza Christina Frade: Approval of the final version of the manuscript; critical review of the literature; drafting and editing of the manuscript.</p><p id="par0055" class="elsevierStylePara elsevierViewall">Gabriela Athayde Amin: Approval of the final version of the manuscript; critical review of the literature; drafting and editing of the manuscript.</p><p id="par0060" class="elsevierStylePara elsevierViewall">Lorena Silva de Carvalho: Approval of the final version of the manuscript; critical review of the literature; drafting and editing of the manuscript.</p><p id="par0065" class="elsevierStylePara elsevierViewall">Lívia Eloi Castro Santos: Critical review of the literature; intellectual participation in the propaedeutic conduct of the studied case.</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Conflicts of interest</span><p id="par0070" class="elsevierStylePara elsevierViewall">None declared.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:4 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Financial support" ] 1 => array:2 [ "identificador" => "sec0010" "titulo" => "Authors' contributions" ] 2 => array:2 [ "identificador" => "sec0015" "titulo" => "Conflicts of interest" ] 3 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2023-02-18" "fechaAceptado" => "2023-03-17" "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "⋆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Study conducted at the Dermatology Service, Centro Universitário do Estado do Pará, Belém, PA, Brazil.</p>" ] ] "multimedia" => array:4 [ 0 => array:8 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1960 "Ancho" => 2508 "Tamanyo" => 609821 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0005" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Right axilla before (A) and after (B) eight weeks of treatment. Considerable reduction in ulcerous-crusted plaques and blisters, with a predominance of residual hyperchromia at the end of treatment.</p>" ] ] 1 => array:8 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 2087 "Ancho" => 2508 "Tamanyo" => 599629 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0010" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Left axilla before (A) and after (B) eight weeks of treatment. Decrease in ulcerous-crusted plaques and blisters, and predominance of residual hyperchromia at the end of treatment.</p>" ] ] 2 => array:8 [ "identificador" => "fig0015" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 1739 "Ancho" => 2508 "Tamanyo" => 403860 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0015" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Abdomen before (A) and after (B) eight weeks of treatment. Considerable reduction in ulcerous-crusted plaques and blisters, and predominance of residual hyperchromia at the end of treatment.</p>" ] ] 3 => array:8 [ "identificador" => "fig0020" "etiqueta" => "Figure 4" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr4.jpeg" "Alto" => 1750 "Ancho" => 1508 "Tamanyo" => 394471 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0020" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Light Microscopy with extensive acantholysis with a “dilapidated brick wall" appearance of the epidermis (Hematoxylin & eosin, ×400).</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:7 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Darier and Hailey-Hailey disease: update 2021" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "D.F. 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 25 | 5 | 30 |
| 2024 October | 168 | 79 | 247 |
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| 2024 March | 434 | 76 | 510 |
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| 2024 January | 83 | 65 | 148 |
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