Frontal fibrosing alopecia in a woman with vulvar erosive lichen planus

Porriño-Bustamante, María Librada; Lázaro-Ochaita, Pablo; Fernández-Pugnaire, María Antonia

doi:10.1016/j.medcle.2020.12.029

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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. 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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×)." ] ] ] "textoCompleto" => "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">1</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).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.<elsevierMultimedia ident="fig0005"></elsevierMultimedia>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.LP is a chronic disorder that can affect the skin, nails, hair and mucoses.<a class="elsevierStyleCrossRef" href="#bib0035">2</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">3</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">2</a> LPV predominantly appears in the 5–6th decade of life,<a class="elsevierStyleCrossRef" href="#bib0045">4</a> and is likely underdiagnosed.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">3</a> Unless LSV, LPV may affect vagina, causing erosions and stenosis.<a class="elsevierStyleCrossRef" href="#bib0040">3</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">2,3</a>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">4</a> Autoimmune disorders are more frequent in eLPV, being thyroid disease the most prevalent one. Moreover, coexistence of LSV and LPV is not rare.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">5</a>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">1</a> An autoimmune hypothesis for the pathogenesis of FFA has been recently proposed,<a class="elsevierStyleCrossRef" href="#bib0030">1</a> pointing the infundibulum melanocytes as an antigenic target. Polyautoimmunity may justify the coexistence of FFA and LPV.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.Funding sourcesNone.Conflict of interestNone declared." "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" => "xack571969" "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" => "(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). 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ISSN: 2387-0206

Launched in 1943, Medicina Clínica is a fortnightly journal aimed at the promotion of clinical research and practice among internal medicine and other specialists. The key characteristics of Medicina Clínica are the scientific and methodological rigor of its manuscripts, the topicality of its contents, and, especially, its practical focus with highly useful information for clinical practice. Medicina Clínica is predominantly interested in publishing original research manuscripts, which are rigorously selected according to their quality, originality, and interest, and also in continued medical education-oriented manuscripts, which are commissioned by the journal to relevant authors (Editorials, Reviews, and Diagnosis and Treatment). These manuscripts contain updated topics with a major clinical or conceptual relevance in modern medicine. The journal adheres to the standards of academic research publications in all aspects including peer-review and ethical principles. Medicina Clínica is included in the General and Internal Medicine category of Thomson Reuters.

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Current Contents/Clinical Medicine, Journal Citation Reports, SCI-Expanded, Index Medicus/Medline, Excerpta Medica/EMBASE, IBECS, IME, MEDES, PASCAL, SCOPUS, SciVerse ScienceDirect

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Impact factor

The Impact Factor measures the average number of citations received in a particular year by papers published in the journal during the two preceding years.

© Clarivate Analytics, Journal Citation Reports 2022

Impact factor 2023

2.6

Citescore

CiteScore measures average citations received per document published.

Citescore 2023

3.1

SJR

SRJ is a prestige metric based on the idea that not all citations are the same. SJR uses a similar algorithm as the Google page rank; it provides a quantitative and qualitative measure of the journal's impact.

SJR 2023

0.371

SNIP

SNIP measures contextual citation impact by wighting citations based on the total number of citations in a subject field.

SNIP 2023

0.511

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Journal Information

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Vol. 157. Issue 11.

Pages e341-e342 (December 2021)

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