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      Liquen escleroso genital pediátrico en niñas: serie de 11 casos Translated title: Pediatric genital lichen sclerosus: a case series of 11 girls

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          Resumen

          Fundamento:

          El liquen escleroso genital disminuye la calidad de vida de las mujeres; el 10-15% de los casos se dan en prepúberes.

          Métodos:

          Estudio descriptivo retrospectivo de las características de las niñas diagnosticadas con liquen escleroso genital en el Hospital Universitario de Navarra (España) entre 2019 y 2022.

          Resultados:

          Se diagnosticaron once niñas entre 4 y 14 años. Fue habitual un retraso diagnóstico tras la aparición de las lesiones de hasta dos años; la niña con cuatro años de retraso presentaba una importante alteración arquitectural vulvar. Todas presentaban lesiones escleróticas típicas en genitales, y dos de ellas también tenían afectación en espalda. Seis pacientes eran asintomáticas, el resto refería prurito y/o dolor. El tratamiento con corticoides tópicos de alta/muy alta potencia logró una buena respuesta parcial, sin remisión completa de las lesiones.

          Conclusión:

          El diagnóstico precoz de liquen escleroso genital es clave para para iniciar el tratamiento lo antes posible y evitar una alteración estructural genital irreversible.

          Abstract

          Background:

          Genital lichen sclerosus decreases the quality of life of women; 10-15% of cases occur in prepubertal girls.

          Methods:

          Retrospective and descriptive study on the characteristics of girls diagnosed with genital lichen sclerosus at the Hospital Universitario de Navarra (Pamplona, Spain) between 2019 and 2022.

          Results:

          Eleven girls aged between 4 and 14 year-old were diagnosed. Frequently, diagnostic delays were up to two years after the appearance of the lesions; the girl with a four-year delay showed a significant vulvar architectural alteration. All cases showed the typical sclerotic lesions on the genital area, and two of them also on the back. While six patients were asymptomatic, the rest reported pruritus and/or pain. Treatment with high/very high potency topical corticosteroids achieved a good partial response, without complete remission of the lesions.

          Conclusion:

          Early diagnosis of genital lichen sclerosus is key to start early treatment, avoiding irreversible genital structural alteration.

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          Most cited references15

          • Record: found
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          British Association of Dermatologists guidelines for the management of lichen sclerosus, 2018.

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            Evidence-based (S3) Guideline on (anogenital) Lichen sclerosus.

            Lichen sclerosus (LS) is an inflammatory skin disease that usually involves the anogenital area. All patients with symptoms or signs suspicious of lichen sclerosus should be seen at least once initially by a physician with a special interest in the disease in order to avoid delay in diagnosis, as early treatment may cure the disease in some and reduce or prevent scarring. The diagnosis is made clinically in most cases. Biopsies should only be performed under certain circumstances. The gold standard for treatment remains potent to very potent topical steroids; however, mild and moderate disease in boys and men may be cured by circumcision. Certain triggers should be avoided. http://www.euroderm.org/images/stories/guidelines/2014/S3-Guideline-on-Lichen-sclerosus.pdf http://www.awmf.org/fachgesellschaften/mitgliedsgesellschaften/visitenkarte/fg/deutsche-gesellschaft-fuer-gynaekologie-und-geburtshilfe-dggg.html.
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              Diagnosis and Treatment of Vulvar Lichen Sclerosus: An Update for Dermatologists.

              Vulvar lichen sclerosus is an important skin disease that is common in women in their 50 s and beyond; however, it can also affect females of any age, including children. If not treated, it has the potential to cause significant and permanent scarring and deformity of the vulvar structure. In addition, if untreated, it is associated with a 2-6% lifetime risk of malignant squamous neoplasia of the vulva. Lichen sclerosus has been considered a difficult to manage condition; however, both serious complications can potentially be prevented with early intervention with topical corticosteroid, suggesting that the course of the disease can be treatment modified.
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                Author and article information

                Journal
                An Sist Sanit Navar
                An Sist Sanit Navar
                assn
                Anales del Sistema Sanitario de Navarra
                Gobierno de Navarra. Departamento de Salud
                1137-6627
                2340-3527
                18 November 2022
                Sep-Dec 2022
                : 45
                : 3
                : e1014
                Affiliations
                [1] originalServicio de Dermatología. Hospital Universitario de Navarra. Servicio Navarro de Salud-Osasunbidea. Pamplona. España. normalizedServicio Navarro de Salud-Osasunbidea orgdiv2Servicio de Dermatología orgdiv1Hospital Universitario de Navarra orgnameServicio Navarro de Salud-Osasunbidea Pamplona, Spain
                Author notes
                [Correspondencia ] Jorge Aróstegui Aguilar. E-mail: jarosteguiaguilar@ 123456gmail.com

                Conflictos de intereses: Los autores declaran no tener conflictos de intereses.

                Financiación: Los autores declaran no haber recibido financiación externa para la realización de este estudio.

                Contribución de autores: Todos los autores han contribuido por igual a la conceptualización, investigación, validación, visualización, redacción del borrador original, y revisión del documento final.

                Author information
                http://orcid.org/0000-0002-4939-2733
                Article
                10.23938/ASSN.1014
                10065031
                36408571
                0ece2bdd-1f1e-4f2e-864a-59289b990fa9

                Este es un artículo publicado en acceso abierto bajo una licencia Creative Commons

                History
                : 26 June 2022
                : 06 August 2022
                : 05 September 2022
                Page count
                Figures: 2, Tables: 1, Equations: 0, References: 12, Pages: 0
                Categories
                Original Breve

                liquen escleroso genital,pediatría,niña,dermatología,glucocorticoides,vulvar lichen sclerosus,pediatrics,female,dermatology,glucocorticoids

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