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.

Extensive bullous lichen sclerosus et atrophicus

Abstract Lichen sclerosus et atrophicus is a chronic disease of unknown etiology characterized by atrophic and sclerotic plaques in both genital and extragenital regions. Extensive bullous lichen sclerosus et atrophicus (BLSA) is a severe variant of the disease with no widely accepted treatment. We present a 63-year-old woman with extensive extragenital, ivory-colored, atrophic plaques on her trunk and extremities and disseminated hemorrhagic bullae. The patient was unsuccessfully treated with standard topical corticosteroid therapy, doxycycline and chloroquine. According to the literature, there is little evidence of the efficacy of doxycycline and hydroxychloroquine in the treatment of BLSA. We report a rare case of extensive BLSA that is unresponsive to these drugs.

Lichen sclerosus associated with localized scleroderma: dermoscopy contribution

Abstract: Lichen sclerosus is an uncommon inflammatory dermatosis with preferential involvement of the urogenital region. The extragenital involvement is uncommon and is characterized by small rounded macules or papules, pearly white in color. The coexistence of lichen sclerosus and scleroderma plaques in most cases with extragenital location has been reported in the literature. We report a case of lichen sclerosus associated with scleroderma in children, highlighting the importance of dermoscopy in diagnosis.

Liquen escleroso extragenital: Presentación de un caso / Lichen scleros extragenital: presentation of a case

El liquen escleroso es una enfermedad crónica inflamatoria de causa desconocida. En hombres su presentación es infrecuente y se observa más comúnmente en la cuarta década de la vida. Asimismo, las lesiones extragenitales son inusuales y asientan más comúnmente en extremidades y tronco. A continuación, presentamos un paciente de sexo masculino adolescente con diagnóstico de liquen escleroso en dorso

Lichen sclerosus is a chronic inflammatory disease of unknown etiology. Appearance in men is unusual and is most seen at fourth decade of life. Also extragenital lesions are rare and most commonly settle in extremities and trunk. A case of a male adolescent patient with lichen sclerosus in trunk is reported.

Bullous and hemorrhagic lichen sclerosus - Case report

Lichen sclerosus is a chronic inflammatory disease, usually located in the genital area. The etiology of lichen sclerosus is multifactorial, with participation of genetic, autoimmune, infectious and hormonal factors. Bullous clinical form stems from hydropic degeneration of the basal membrane, constituting a less frequent variant of the disease. In this work, we report the case of a female patient, 55 years old, who in the last three years presented whitish plaques, with horny spikes, located on back and arms. Some of these lesions evolved with hemorrhagic blisters, which after histopathological examination confirmed the diagnosis of bullous and hemorrhagic lichen sclerosus. The patient was treated with high-potency topical corticosteroid for two months, resulting in remission of bullous and hemorrhagic lesions.

Liquen escleroso y atrófico: revisión de una dermatosis con múltiples manifestaciones / Lichen sclerosus and atrophic: review of a dermatosis with multiple manifestations

El liquen escleroso y atrófico (LEA) es una enfermedad inflamatoria crónica poco frecuente, de causa desconocida, con tendencia a la atrofia epidérmica y cicatrización destructiva. Predomina en mujeres, en la región anogenital, asociándose a un importante deterioro funcional y, en ocasiones, transformación maligna a carcinoma espinocelular. El tratamiento de elección es aún controvertido, siendo los corticoides tópicos de alta potencia y los inhibidores tópicos de la calcineurina los más utilizados. Se presentan cuatro casos clínicos de LEA; uno en una niña de 8 años, con una placa blanquecina atrófica localizada en tórax anterior; un segundo caso, un paciente de sexo masculino de 31 años con una placa blanquecina atrófica localizada en el glande, prepucio y cuerpo del pene; un tercer caso, un paciente de sexo masculino de 24 años con pápulas blanquecinas de 1 mm de diámetro, localizadas en el cuerpo del pene; y finalmente, una paciente de sexo femenino de 53 años con placas blanquecinas, atróficas e induradas en la axila derecha. Todos con hallazgos histopatológicos característicos que permitieron confirmar el diagnóstico de LEA. A partir de estos casos destacamos las diferentes localizaciones y edades de presentación que puede tener esta enfermedad junto con la importancia de un diagnóstico e inicio precoz del tratamiento. Esta revisión tiene como objetivo actualizar los conocimientos sobre los datos demográficos, clínicos, fisiopatológicos y terapéuticos en torno a LEA. Para ello, se realizó una búsqueda exhaustiva de la literatura utilizando los buscadores de PubMed y la Colaboración Cochrane. Resultados de la búsqueda incluyen bibliografía publicada hasta julio de 2014.

Lichen sclerosus et atrophicus (LSA) is an uncommon chronic inflammatory disease of unknown cause, prone to produce epidermal atrophy and destructive scarring. It predominates in women, in the anogenital region, usually associated with significant functional impairment and sometimes malignant transformation to squamous cell carcinoma (SCC). The treatment of choice is still controversial, with topical high potency steroids and topical calcineurin inhibitors being actually the most used. Four clinical cases are presented: one from an 8 year-old girl with a whitish atrophic plaque located on the chest; another is a male patient, aged 31, with a whitish atrophic plaque located on the glans, foreskin and body of the penis; a third case, 24 year-old male, with whitish papules of 1 mm in diameter located on the body of the penis and; finally, a female patient aged 53, with white atrophic and indurated plaques at the right axilla. All of them had characteristic histopathologic findings, confirming the diagnosis of LSA. From these cases we pretend to highlight the different locations and ages of presentation of LSA, and the importance of an early diagnosis and treatment. This review update the current understanding of the demographic, clinical, pathogenic and therapeutic data on LSA. For this, a comprehensive search of the literature was conducted using PubMed and Cochrane Library. Search results include published references until july 2014.

Case for diagnosis / Caso para diagnostico

Linear Darier is a rare variant of Darier's Disease. The keratotic papules follow Blaschko's lines, characterizing the cutaneous mosaicism. We report the case of a 42-year-old woman who presented with small, linearly distributed, reddish-brown papules on the left mammary region, from the left flank to the hypogastrium and on the pretibial area of the left leg. Histologically, suprabasal acantholysis and dyskeratotic cells were seen, confirming the diagnosis.

Darier linear é uma variante rara da Doença de Darier. As pápulas ceratósicas se distribuem seguindo as linhas de Blaschko, caracterizando o mosaicismo do envolvimento cutâneo. Relatamos o caso de uma mulher de 42 anos que apresentava pápulas acastanhadas, distribuídas linearmente na região mamária esquerda, do flanco esquerdo ao hipogástrio e na região prétibial esquerda. Histologicamente, foram observadas acantólise suprabasal e células disceratósicas, confirmando a suposição diagnóstica.

Clinicopathological correlation of acquired hypopigmentary disorders.

Acquired hypopigmentary disorders comprise a significant group of disorders that affect Indians and Asians. The pigment disturbance in darker skin individuals can be very distressing to the patient and the family. These disorders cover a wide array of pathologies including infections, autoimmune processes, lymphoproliferative disorders, and sclerosing diseases. Histological diagnosis is particularly important because treatments for these diseases are varied and specific. This review will focus on histopathological diagnosis based on clinicopathological correlation for commonly encountered disorders such as leprosy, vitiligo, lichen sclerosus, pityriasis alba (PA), and pityriasis versicolor (PV). Atypical or uncommon clinical presentation of classic diseases such as hypopigmented mycosis fungoides (HMF) and hypopigmented sarcoidosis are also included.

Scanning electron microscopy of lichen sclerosus / Microscopia eletrônica de varredura do líquen escleroso

Lichen sclerosus is an acquired inflammatory condition characterized by whitish fibrotic plaques, with a predilection for the genital skin. We performed scanning electron microscopy of the dermis from a lesion of lichen sclerosus. Normal collagen fibers could be easily found in deeper layers of the specimen, as well as the transition to pathologic area, which seems homogenized. With higher magnifications in this transitional area collagen fibers are adherent to each other, and with very high magnifications a pearl chain aspect became evident along the collagen fibers. In the superficial dermis this homogenization is even more evident, collagen fibers are packed together and round structures are also observed. Rupture of collagen fibers and inflammatory cells were not found. These autoimmune changes of the extracellular matrix lead to the aggregation of immune complexes and/or changed matrix proteins along the collagen fibers, the reason why they seem hyalinized when examined by light microscopy.

O líquen escleroso é uma afecção inflamatória caracterizada por placas esbranquiçadas fibróticas ocorrendo preferentemente na pele genital. Realizamos exame com microscópio eletrônico de varredura da derme de uma lesão de líquen escleroso. Ao exame as fibras colágenas normais puderam ser facilmente identificadas, bem como a transição para a área alterada, a qual aparece homogeneizada. Nessa área as fibras parecem aderidas umas às outras e com aspecto em colar de pérolas. Na derme superficial essa homogeneização é ainda mais evidente, com as fibras bem aderidas e ainda com as estruturas arredondadas. Ruptura das fibras e células inflamatórias não foram observadas. Os fenômenos autoimunes que ocorrem na matriz extracelular nessa enfermidade devem levar à agregação de imunocomplexos e/ou proteínas alteradas nas fibras colágenas, por essa razão elas aparecem hialinizadas na microscopia óptica.

Case for diagnosis / Caso para diagnóstico

Lichen sclerosus is a chronic inflammatory mucocutaneous disorder of unknown etiology that most commonly affects the female genitalia. Cutaneous involvement with nonhaemorrhagic bullous is very unusual. We describe a case of bullous lichen sclerosus.

Líquen escleroso é uma doença mucocutânea inflamatória, crônica, de etiologia desconhecida, que afeta mais comumente a genitália feminina. O envolvimento cutâneo com bolhas não hemorrágicas é muito raro. Descrevemos um caso de líquen escleroso bolhoso.

Líquen escleroso e atrófico acral: relato de caso / Acral lichen sclerosus et atrophicus: case report

O líquen escleroso e atrófico é uma doença crônica da pele e mucosas, que, usualmente, afeta a área genital de mulheres. Poucos casos de líquen escleroso e atrófico, confinados nos pés e/ou mãos, têm sido relatados. Relatamos um caso de líquen escleroso e atrófico envolvendo as extremidades e,posteriormente, a área perigenital.

Lichen sclerosus et atrophicus is a chronic disorder of the skin and mucosal surfaces, most commonly affecting the female genitalia. Few cases of lichen sclerosus et atrophicus confined to the feet and/or hands have been reported. We report a case of lichen sclerosus et atrophicus involving first the extremities and then the perigenital area.

Koebnerization in a woman with extragenital lichen sclerosus

Lichen sclerosus [LS] is a chronic benign inflammatory disorder of skin and mucosa which affects patients of all age groups, sex and race, particularly caucasian prepubertal girls and postmenopausal women. The etiology of LS is unknown, but it has been suggested that genetic predisposition to inflammatory disorders, immunological constitutions, hormonal influences and local factors might play a role. Extragenital LS are most common on the neck, shoulders and upper portion of the trunk. It is generally asymptomatic, but occasionally pruritic. The Koebner sign is a well-known diagnostic marker in LS. Typical lesions have been noted to occur following surgical operations, infections, rubbing of skin, in old thermal burn scar, sexual abuse and under tight clothing. We describe a 25-year-old Iranian woman presented with a 2-month history of extragenital LS. The multiple classical lesions of disease were presented over her neck, shoulders, upper part of trunk, the medial area of her thighs and feet at the dorsal site. The diagnosis of LS was confirmed by histological findings. Beside LS classical lesions on her back, linear lesions including Koebner phenomenon were observed at the sites of rubbing and itching of skin

[ epidemio clinical of lichen sclerosus about 31 cases]

Lichen sclerosus is a chronic inflammatory mucocutaneous disease. Through a retrospective study of all patients with Lichen sclerosus followed between 1997 and 2005 at the dermatology department of La Rabta hospital, we outline the epidemio-clinical aspects of this entity. There were 29 female and two male. Two patients were child. The mean age at the occurrence of adult lichen sclerosus was 51, 5 years. The site of the lesions was perineum and genital in 26 cases [one male and 25 female]. Extragenital involvement was present in 10 patients. All patients underwent topical corticosteroid therapy. Architectural changes of vulva were noted in 7 cases. An epidermoid carcinoma occurred in five patients. The male Lichen sclerosus seem to be rare in our study. It is probably explained by the protective role of the circumcision which is practised in all male children in our country. In our study, the risk of malignant transformation of genital Lichen sclerosus is estimated at 19, 2%. It is lightly more than mentioned in the literature. This may be related to the cofactor role of l-IPV and to an insufficient treatment adherence

Dermatoses vulvares: revisão de tema / Vulvar dermatosis: review of the literature

Em 1989, após amplo debate entre ginecologistas, dermatologistas e anatomopatologistas, a Sociedade Internacional para Estudo das Doenças da Vulva (International Society for the Study of Vulvar Disease - ISSVD) definiu uma clasificação baseada nas características histopatológicas das lesões vulvares, que foi aceita pelas especialidades, bem como por diferentes organizações internacionais. As dermatoses vulvares foram classificadas como Hiperplasia de Células Escamosas, Líquen Escleroso e outras Dermatoses. Os autores fizeram uma revisão bibliográfica, buscando sintetizar e harmonizar diferentes aspectos das patologias segundo a óptica de diferentes autores. Além da Hiperplasia de Células Escamosas e Líquen Escleroso, foram estudados Líquen Plano, Líquen Simples Crônico, Psoríase, Intertrigo, Eczema e Dermatite Seborréica, dermatoses mais comuns no ambulatório de atuação dos profissionais em Ginecologia.