Practical Dermoscopy
Candy-Cane Nails
Uñas «en bastón de caramelo»
J. Sánchez-Bernal
, M. Álvarez-Salafranca, M. Ara-Martín
Corresponding author
Servicio de Dermatología del Hospital Clínico Universitario Lozano Blesa, Zaragoza
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"apellidos" => "Hussein" ] ] ] ] ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S1578219021001736?idApp=UINPBA000044" "url" => "/15782190/0000011200000008/v2_202110201006/S1578219021001736/v2_202110201006/en/main.assets" ] "en" => array:15 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Practical Dermoscopy</span>" "titulo" => "Candy-Cane Nails" "tieneTextoCompleto" => true "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "755" "paginaFinal" => "756" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "J. Sánchez-Bernal, M. Álvarez-Salafranca, M. Ara-Martín" "autores" => array:3 [ 0 => array:4 [ "nombre" => "J." "apellidos" => "Sánchez-Bernal" "email" => array:1 [ 0 => "jsanchezbe@salud.aragon.es" ] "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "*" "identificador" => "cor0005" ] ] ] 1 => array:2 [ "nombre" => "M." "apellidos" => "Álvarez-Salafranca" ] 2 => array:2 [ "nombre" => "M." "apellidos" => "Ara-Martín" ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Servicio de Dermatología del Hospital Clínico Universitario Lozano Blesa, Zaragoza" "identificador" => "aff0005" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Uñas «en bastón de caramelo»" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:8 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 2341 "Ancho" => 1591 "Tamanyo" => 644908 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0005" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A, Poorly delimited, fleshy erythematous plaques in the inguinal fold. B, Lesions on all fingernails.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Case Description</span><p id="par0005" class="elsevierStylePara elsevierViewall">A 29-year-old man was seen for lesions in the inguinal, intergluteal folds that had appeared 2 months earlier, without other associated lesions of the skin or mucosa-f. In addition (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>A), he presented lesions of the nails that he said had been present since the age of 15 (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>B). Interestingly, he reported that his mother also had similar skin and nail lesions that had appeared during adolescence.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">What Is Your Diagnosis?</span><p id="par0010" class="elsevierStylePara elsevierViewall">Darier disease with exclusively flexural involvement.</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Comment</span><p id="par0015" class="elsevierStylePara elsevierViewall">Polarized light dermoscopy revealed alternating longitudinal bands of leukonychia and erythronychia associated with subungual hyperkeratosis, splinter hemorrhage, and a V-shaped notch at the distal edge of the nail plate (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>). Cutaneous histology revealed the presence of dyskeratosis and suprabasal acantholysis. A genetic study of the patient and his mother detected a mutation in the <span class="elsevierStyleItalic">ATP2A2</span> gene, confirming the suspected diagnosis of Darier disease.</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Darier disease is a rare genodermatosis that is inherited in an autosomal dominant pattern and is caused by mutations in <span class="elsevierStyleItalic">ATP2A2</span>, which encodes a protein involved in the regulation of calcium channels.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> Characteristic findings include the presence of hyperkeratotic papules, which have a seborrheic distribution and are often macerated and foul smelling when located in intertriginous areas, as well as palmar pitting, white papules in the oral mucosa, and dystrophic nails.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,2</span></a> Onset usually occurs at puberty and the disease follows a chronic course, with exacerbations induced by infections, sun or heat exposure, and friction.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Fingernail involvement is described in 92–95% of patients,<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> in which the presence of alternating longitudinal white (linear leukonychia) and red (linear erythronychia) bands (candy-cane pattern) is pathognomonic.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,3</span></a> Longitudinal striae, subungual hyperkeratosis, splinter hemorrhages, and V-shaped notches in the distal free edge of the nail have also been described.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,3,4</span></a> Generally, involvement is polydactylous: the number of affected fingernails ranges from 2 to 5, while toenails tend to be less frequently affected.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">These nail alterations can be key in establishing the differential diagnosis with other dermatoses with similar cutaneous manifestations, including seborrheic dermatitis and Grover disease, in which nail involvement is absent, and Hailey–Hailey disease, in which Burge described characteristic multiple longitudinal bands of leukonychia, predominantly affecting the thumbs.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">This dermoscopic pattern should not be confused with other types of leukonychia or erythronychia that occur in other diseases. For example, onychopapilloma can present as a single longitudinal band of leukonychia or erythronychia and some glomus tumors present as a single longitudinal band of erythronychia that does not reach the distal border.<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3,4,6</span></a> Darier disease should also be distinguished from transverse leukonychia, whether true (trauma-associated) or apparent (generally associated with systemic diseases),<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3,4</span></a> and, above all, from other causes of polydactylous longitudinal erythronychia such as lichen planus, primary amyloidosis, graft-versus-host disease, and acantholytic epidermolysis bullosa.<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3,6</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">Given that the majority of patients with Darier disease present nail alterations,<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> onychoscopy may be useful to facilitate diagnosis in doubtful cases such as that described here, in which flexural involvement and the absence of other skin lesions would suggest a diagnosis of Hailey–Hailey disease, and in patients with no relevant family history.</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Conflicts of Interest</span><p id="par0040" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:5 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Case Description" ] 1 => array:2 [ "identificador" => "sec0010" "titulo" => "What Is Your Diagnosis?" ] 2 => array:2 [ "identificador" => "sec0015" "titulo" => "Comment" ] 3 => array:2 [ "identificador" => "sec0020" "titulo" => "Conflicts of Interest" ] 4 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Sánchez-Bernal J, Álvarez-Salafranca M, Ara-Martín M. Uñas «en bastón de caramelo». Actas Dermosifiliogr. 2021. <span class="elsevierStyleInterRef" id="intr0005" href="https://doi.org/10.1016/j.ad.2019.10.014">https://doi.org/10.1016/j.ad.2019.10.014</span></p>" ] ] "multimedia" => array:2 [ 0 => array:8 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 2341 "Ancho" => 1591 "Tamanyo" => 644908 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0005" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A, Poorly delimited, fleshy erythematous plaques in the inguinal fold. B, Lesions on all fingernails.</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" => 1412 "Ancho" => 1591 "Tamanyo" => 191475 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0010" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Onychoscopy revealing longitudinal, alternating bands of erythronychia and leukonychia (black arrow), splinter hemorrhage beneath the central portion of the lamina (blue arrow), a V-shaped notch at the distal edge of the nail plate (red arrow), and subungual hyperkeratosis (yellow arrow).</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:6 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Darier disease: candy-cane nails and hyperkeratotic papules" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "P. 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