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Frontal fibrosing alopecia in a woman with vulvar erosive lichen planus
Alopecia frontal fibrosante en una mujer con liquen plano erosivo vulvar
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=> "María Librada Porriño-Bustamante, Pablo Lázaro-Ochaita, María Antonia Fernández-Pugnaire" "autores" => array:3 [ 0 => array:4 [ "nombre" => "María Librada" "apellidos" => "Porriño-Bustamante" "email" => array:1 [ 0 => "mporrinobustamante@gmail.com" ] "referencia" => array:3 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] 2 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "Pablo" "apellidos" => "Lázaro-Ochaita" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "María Antonia" "apellidos" => "Fernández-Pugnaire" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "aff0015" ] ] ] ] "afiliaciones" => array:3 [ 0 => array:3 [ "entidad" => "Servicio de Dermatología, Hospital Universitario La Zarzuela, Madrid, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Universidad de Granada, Granada, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Servicio de Dermatología, Hospital Universitario San Cecilio, Granada, Spain" "etiqueta" => "c" "identificador" => "aff0015" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Alopecia frontal fibrosante en una mujer con liquen plano erosivo vulvar" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1381 "Ancho" => 1505 "Tamanyo" => 311056 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">(a) Red erosion on the inner side of left minor labia, surrounded by a whitish area (arrow). A small whitish area is also noted on the inner aspect of right minor labia (asterisk). (b) Small scarring alopecic area placed in the right fronto-temporal side (asterisk), with subtle loss of density in the right sideburn. (c) No frontal hairline recession. (d) Dermoscopy showed loss of follicular openings (asterisk) with subtle perifollicular hyperkeratosis (arrows), and a whitish background (Dermlite 2PRO HR, polarized light, 10×).</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Frontal fibrosing alopecia (FFA) is a scarring alopecia, considered by some as a variant of lichen planopilaris, although it is questionable. Autoimmune conditions have been related to FFA, such as thyroid disorders and vitiligo.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> Regarding other forms of lichen planus (LP), FFA has been associated to LP pigmentosus, lichen planopilaris (LPP) cutaneous and mucous LP, and lichen scleroatrophicus vulvae (LSV).</p><p id="par0010" class="elsevierStylePara elsevierViewall">A 54-year-old woman presented with a one-year history of flares of vulvar pruritus, pain and spotting. She had started menopause three years before. A red erosive area in the vestibule, with a whitish surrounding area, and whitish areas in the minor labia (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>a) were noted. A biopsy confirmed the diagnosis of erosive lichen planus of the vulva (eLPV). Physical examination revealed eyebrow alopecia. Treatment with topical prostaglandins and tacrolimus was prescribed for eyebrows, and hydrocortisone vaginal suppositories, and tacrolimus-clobetasol cream for eLPV.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Two years later, a scarring alopecic area was noted in her right fronto-temporal side, and a subtle decreased of hair density in the right sideburn (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>b). Frontal hairline was preserved (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>c). Dermoscopy showed follicles with one hair, follicular hyperkeratosis and transparent proximal hair emergence (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>d). Diagnosis of FFA was made. Eyebrow alopecia had advanced and she had undergone micropigmentation. Treatment with oral finasteride, topical tacrolimus and periodical intralesional triamcinolone was initiated. One year later, she remains stable and asymptomatic. The patient signed previously an informed consent for the use of off-label medications.</p><p id="par0020" class="elsevierStylePara elsevierViewall">LP is a chronic disorder that can affect the skin, nails, hair and mucoses.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">2</span></a> LPV is less frequent than LSV, and the commonest form is the erosive one, over the papulosquamous and hypertrophic types.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">3</span></a> The oral mucosa is the commonest site of involvement of erosive LP, followed by genital mucosa, although up to 68% of women with eLPV have oral LP.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">2</span></a> LPV predominantly appears in the 5–6th decade of life,<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a> and is likely underdiagnosed.</p><p id="par0025" class="elsevierStylePara elsevierViewall">eLPV presents as bright red areas and erosions with a white lacy edge, often placed on the medial aspect of the minor labia and vaginal orifice. Advanced LPV can distort the vulvar architecture, similar to that in LSV, with agglutination of the minor labia, clitoris phimosis and introital narrowing.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">3</span></a> Unless LSV, LPV may affect vagina, causing erosions and stenosis.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">3</span></a> eVLP usually produces pain, itch or burning, and may associate dyspareunia and vaginal discharge if the vagina is affected.<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">2,3</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">LP is thought to be a T-cell mediated autoimmune disease. The autoimmune phenotype of the LPV and LSV is supported by the demonstration of increased levels of Th1-specific cytokines, dense T-cell infiltrate, and enhanced microRNA-155 expression.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a> Autoimmune disorders are more frequent in eLPV, being thyroid disease the most prevalent one. Moreover, coexistence of LSV and LPV is not rare.</p><p id="par0035" class="elsevierStylePara elsevierViewall">Chew et al. found that 16 out of 83 of patients with LPV had LPP (8/16) or FFA (8/16). All patients with FFA had oral LP, and 7 of them had the erosive variant of LPV.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">5</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">Patients with an autoimmune disease are prone to develop another one, because of the similar genetic and environmental factors among them; it is called polyautoimmunity.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> An autoimmune hypothesis for the pathogenesis of FFA has been recently proposed,<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> pointing the infundibulum melanocytes as an antigenic target. Polyautoimmunity may justify the coexistence of FFA and LPV.</p><p id="par0045" class="elsevierStylePara elsevierViewall">In conclusion, we report a woman with eLPV and eyebrow alopecia, who later developed a scarring alopecic patch in the context of FFA. Further studies are needed to clarify the pathogenesis of both entities. As untreated eLPV and LSV may produce sequels, patients with FFA or LPP may be explored properly with the aim of making an early diagnosis of possible accompanying vulvar diseases, not only for the cutaneous and oral conditions.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Funding sources</span><p id="par0050" class="elsevierStylePara elsevierViewall">None.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Conflict of interest</span><p id="par0055" class="elsevierStylePara elsevierViewall">None declared.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:4 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Funding sources" ] 1 => array:2 [ "identificador" => "sec0010" "titulo" => "Conflict of interest" ] 2 => array:2 [ "identificador" => "xack569232" "titulo" => "Acknowledgements" ] 3 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "multimedia" => array:1 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1381 "Ancho" => 1505 "Tamanyo" => 311056 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">(a) Red erosion on the inner side of left minor labia, surrounded by a whitish area (arrow). A small whitish area is also noted on the inner aspect of right minor labia (asterisk). (b) Small scarring alopecic area placed in the right fronto-temporal side (asterisk), with subtle loss of density in the right sideburn. (c) No frontal hairline recession. (d) Dermoscopy showed loss of follicular openings (asterisk) with subtle perifollicular hyperkeratosis (arrows), and a whitish background (Dermlite 2PRO HR, polarized light, 10×).</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:5 [ 0 => array:3 [ "identificador" => "bib0030" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Frontal fibrosing alopecia: a new autoimmune entity?" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:6 [ 0 => "J.E. Garcia-Robledo" 1 => "C.C. Aragón" 2 => "I. Nieto-Aristizábal" 3 => "S. 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