Liquen escleroso genital y extragenital en la infancia: a propósito de un caso clínico / Lichen sclerosus in childhood

El liquen escleroso es una dermatosis inflamatoria crónica de piel y semimucosas que afecta princi-palmente vulva y región perianal, la localización extragenital vista sólo en 15 a 20% de los casos es rara en la infancia. La presentación más frecuente es la anogenital con pápulo-placas blanco marfil, atrofia, erosiones, púrpura y esclerosis. En mujeres afecta simétricamente zona interna de vulva, pe-rianal y periné adquiriendo forma típica en ocho, sin afectar vagina o himen. El liquen escleroso ex-tragenital se localiza principalmente en el dorso, cuello, periumbilical, muñecas y axilas con lesiones similares a las descriptas. Presentamos el caso clínico de una escolar de 11 años, sin patologías previas, que consulta por der-matosis situada en piel y mucosas topografiada en tronco y genitales. En piel, zona escapular bilate-ral, se observan placas blanco-nacaradas de cen-tro atrófico, sin esclerosis palpable. En mucosas, afectaba labios mayores, menores y zona perianal en forma de ocho, con atrofia y coloración blanco-brillosa. Refiere un año de evolución, con prurito vulvar y constipación. Se realiza diagnóstico de li-quen escleroso genital y extragenital y tratamiento con buena evolución.La importancia de conocer su diagnóstico radica en que es una patología que afecta la calidad de vida de los pacientes, por su sintomatología, su po-sible repercusión sexual y urinaria, y su potencial riesgo de carcinoma espinocelular. Es importante tener un alto índice de sospecha, ya que, debido a su baja frecuencia en la edad pediátrica, muchas veces el diagnóstico es tardío.

Lichen sclerosus is a chronic inflammatory der-matosis of skin and semi-mucosae, important for its sexual and urinary repercussion, and its poten-tial risk of squamous cell carcinoma.Mainly affects vulva and perianal region, extrage-nital localization seen only in 15 to 20% of cases is rare in childhood. The most frequent presenta-tion is anogenital with white ivory papule-plates, atrophy, erosions, purpura and sclerosis. In women it affects the inner area of the vulva, peria-nal and perineum, acquiring a typical shape in eight, without affecting the vagina or hymen. The extragenital sclerosus lichen is located mainly on the back, neck, periumbilical, wrists and armpits with lesions similar to those described.We present the case of a schoolgirl of 11 years, without previous pathologies, who consulted for dermatosis located on the skin and mucous mem-branes on the trunk and genitals. In skin, bilateral scapular area, white-nacreous plates of atrophic center are observed, without palpable sclerosis. In mucous membranes, it affected the labia mi-nora, majora and perianal zone in eight form, with atrophy and white-shiny coloration. She referred a year of evolution, with vulvar pruritus and constipation. Genital and extragenital lichen sclerosus diagnosis was made and had a good evolution with treatment.

Liquen esclero atrófico extragenital / Extra-Genital Atrophic Lichen Sclerosus

El Liquen esclero atrófico es una enfermedad inflamatoria crónica de la piel que afecta la epidermis y la dermis, la causa es desconocida, aunque se considera que la génesis es multifactorial. Los principales factores con los que se ha relacionado, incluyen los genéticos, inmunológicos, infecciosos, traumáticos y hormonales. Se presenta el caso de un paciente masculino de 24 años de edad con diagnóstico de Liquen esclero atrófico extra genital, con antecedentes de asma bronquial desde la infancia, hace 5 años presenta múltiples lesiones en placas acrómicas, de aspecto nacarado, algunas atróficas en su región central, asintomáticas(AU)

Atrophic Lichen Sclerosus is a chronic inflammatory disease of the skin that affects the epidermis and dermis, the cause is unknown, although the genesis is considered to be multifactorial. The main associated factors have been genetic, immunological, infectious, traumatic and hormonal. A case of a 24-year-old male patient is presented here. He has a diagnosis of extra-genital atrophic lichen sclerosus, with a history of bronchial asthma since infancy. For 5 years, this patient has multiple lesions on acrylic plaques, pearly in appearance, some atrophic in the central region, asymptomatic(AU)

O tratamento bem-sucedido com fototerapia UVB banda estreita em um caso raro de líquen escleroso extragenital generalizado / Successful treatment of a rare case of widespread extragenital lichen sclerosus with narrow band UVB phototherapy

O líquen escleroso é patologia incomum acometendo tanto região genital quanto extragenital, constituindo apresentação atípica da doença. O uso de corticosteroides tópicos é considerado primeira linha para tratamento da doença genital. Lesões extragenitais são menos sensíveis a essa terapêutica. Relata-se caso de líquen escleroso em paciente do sexo feminino, de 82 anos, com envolvimento extragenital generalizado e tratamento bem-sucedido com fototerapia UVB de banda estreita. O líquen escleroso é dermatose inflamatória crônica, e as formas disseminadas da doença são raras e sem consenso em relação ao tratamento da forma extragenital. A opção pela terapia com UVB de banda estreita baseou-se nos relatos da literatura.

Lichen sclerosus is an uncommon condition affecting both the genital and extragenital region, the latter constituting an atypical occurrence of the disorder. The use of topical corticosteroids is considered the first-line treatment of this genital disease. Extragenital lesions are less sensitive to this therapy. The present study describes a case of lichen sclerosus in an 82-year-old female patient, with widespread extragenital involvement and successful treatment with narrow band UVB phototherapy. Lichen sclerosus is a chronic inflammatory dermatosis and disseminated forms of the condition are rare, with an absence of consensus on the treatment of the extragenital form. The choice for narrow band UVB therapy was based on the literature.

Lichen sclereux genital

Lichen sclerosis is a chronic inflammatory mucocutaneous, disease which origin remains unknown. Its prevalence ranges from one in 300 to one in 1000 of all patients referred to a dermatology clinic in the seventeeth. Through the analysis of a hospital survey, we outline the epidemio-clinical aspects of this dermatosis. Over a 19-year period [1984-2002], we have conducted a retrospective and monocentric study of all patients with genital lichen sclerosis were examined at the dermatology department of Habib Thameur hospital. Thirty-four patients suffered from lichen sclerosis. There were 33 female and only one male [sex-ratio: 0.03]. All patients underwent topical corticosteroid therapy [level I, II or IV]. The recovery rate of lichen sc1erosus was about 20% [7/34]. An epidermoid carcinoma occurred in three patients. The frequency of lichen sc1erosus in our study is estimated at 1,8 new cases per year. This frequency is probably under-estimated because of some patients' reluctance to seek help. A relatively low recovery rate of genital lichen sclerosis was found in our study. This may be related to an inadequate follow up added to an insufficient treatment adherence

Liquen escleroso: a propósito de un caso / Lichen sclerosus: apropos of a case

Se presenta la evolución de un liquen escleroso extragenital en una paciente diabética, de 67 años, con discordancia inicial entre la clínica y la histopatología, el cual posteriormente se complicó con un carcinoma espinocelular vulvar. Se realiza una revisión del tema y de la conducta terapéutica, especialmente en mujeres.