Coexistence of chronic cutaneous lupus erythematosus and frontal fibrosing alopecia

Abstract: Lupus erythemathosus is a chronic, relapsing disease with acute, subacute, and chronic lesions. Effluvium telogen occurs in the setting of systemic activity of the disease, and cicatricial alopecia results from discoid lesionsin on the scalp. Other types of alopecia, like alopecia areata, may rarely be found in lupus. Frontal fibrosing alopecia is characterized by frontotemporal hairline recession and eybrow loss. Histophatologically, it cannot be differentiated from lichen planopilaris.It is controversial whether frontal fibrosing alopecia is a subtype of lichen planopilaris.. A pacient with chronic lichenoid lupus erythematosus is described with clinical, histophatological and dermoscopic features of frontal fibrosing alopecia.We have not been able to find in the literature cases of frontal fibrosing alopecia as a clinical manifestation of lupus.

Segmental lichen aureus: an uncommon presentation

Abstract: Pigmented purpuric dermatoses are chronic vascular inflammatory conditions characterized by the presence of pigmented macules. Among its different presentations, lichen aureus is distinguished by the lichenoid conformation of its plaques and the predilection for lower limb involvement. Its segmented form is rare and difficult to control, especially in cases of symptomatic lesions. We report a rare case of segmental lichen aureus with six years of evolution associated with light itching. We also discuss the main therapeutic approaches to control the disease.

Liquen aureus zosteriforme: Caso dínico pediatrico / Zosteriform lichen aureus: Pediatric clinical case

El liquen aureus es una dermatosis purpúrica pigmentaria infrecuente. Presentamos un caso inusual por su edad pediátrica y por la presencia de lesiones múltiples con patrón zosteriforme. Se trata de un niño de 10 años con máculas parduzcas, menores de 1 cm, con bordes definidos y superficie liquenificada, en la cara interna de la pierna izquierda con distribución zosteriforme, asintomáticas, de tres meses de evolución. La biopsia mostró infiltrado en banda en la dermis superior con linfocitos, histiocitos, hematíes y hemosiderina. Se diagnosticó liquen aureus zosteriforme y se trató con furoato de mometasona tópico durante 3 semanas con aclaramiento parcial. Las lesiones permanecen 2 años después y han aparecido nuevas en el tobillo ipsilateral. Debemos plantearnos el diagnóstico diferencial con otras dermatosis purpúricas pigmentarias crónicas y la variante purpúrica pigmentaria de la micosis fungoide. Existen muchas opciones terapéuticas e, incluso sin tratamiento, suelen desaparecer, por lo que son necesarios estudios que recopilen más evidencias.

ABSTRACT Lichen aureus is a rare pigmented purpuric dermatosis. We present anunusual case because of the pediatric age and the great number of lesions with zosteriform distribution. He is a 10-year-old boy, with a brownish, smaller than 1 cm, sharp edges, lichenified surface, asymptomatic macule, over the inner aspect of the left leg with a zosteriform distribution. The histology showed a band-like inflammatory infiltrate in the superficial dermis, composed of lymphocytes, histiocytes, erythrocytes and haemosiderin. He was diagnosed with zosteriform lichen aureus and was treated with topical mometasone furoate during 3 weeks resulting in partial lightening of the macules. Lesions have remained 2 years later, and new ones have appeared in the ipsilateral ankle. We must consider differential diagnosis with other pigmented purpuric dermatitis and pigmented purpuric mycosis fungoides. There are many therapeutic options and it tends to disappear spontaneously, so new studies are necessary

Necrolytic acral erythema: a rare skin disease associated with hepatitis C virus infection

Abstract: Necrolytic acral erythema is a rare skin disease associated with hepatitis C virus infection. We report a case of a 31-year-old woman with hepatitis C virus infection and decreased zinc serum level. Physical examination revealed scaly, lichenified plaques, well-demarcated with an erythematous peripheral rim located on the lower limbs. After blood transfusion and oral zinc supplementation the patient presented an improvement of lesions.

Agminate lichen aureus / Liquen aureus agminado

Lichen aureus is a rare variant of pigmented purpura, with a chronic and benign course. It is generally asymptomatic and often occurs in the lower limbs, presenting as erythematous brownish, coppery or golden macules and/or papules. The diagnosis is based on clinical and histopathological findings. The dermatoscopic pattern has been considered a useful tool in diagnosis presumption. We describe a case with a confluent morphological pattern, called agminate lichen aureus.

O líquen aureus é uma variante rara das púrpuras pigmentares, com evolução crônica e benigna. A maioria é assintomática e predomina nos membros inferiores. O quadro clínico é constituído por máculas e/ou pápulas eritemato-acastanhadas, acobreadas ou douradas. O diagnóstico é clínico e histopatológico, porém o padrão dermatoscópico vem sendo uma ferramenta útil na presunção do diagnóstico. Descrevemos um caso com um padrão morfológico em que a lesão é confluente, sendo denominado líquen aureus agminado.

Chronic graft-versus-host disease: clinical presentation of multiple lesions of lichenoid and atrophic pattern / Doenca enxerto contra hospedeiro cronica: apresentacao clinica com inumeras lesoes de padrao liquenoide e de carater atrofico

Graft-versus-host disease is observed mainly in recipients of hematopoietic cell transplantation and is expressed by cutaneous or systemic signals and symptoms. Graft-versus-host disease is clinically classified as acute or chronic. Chronic Graft-versus-host disease occurs in up to 70% of hematopoietic cell transplanted patients and its clinical manifestations have important impact on morbidity and quality of life. The authors report an expressive cutaneous, oral and adnexal involvement in a patient with chronic Graft-versus-host disease with multiple lesions of lichenoid and atrophic pattern.

Doença enxerto contra hospedeiro é observada principalmente em pacientes transplantados de células de origem hematopoiéticas e se expressa por sinais e sintomas cutâneos ou sistêmicos. Clinicamente, a Doença enxerto contra hospedeiro é classificada em aguda ou crônica. As do tipo crônico ocorrem em até 70% dos pacientes e suas manifestações têm impacto importante na morbidade e na qualidade de vida. Os autores relatam caso com extenso comprometimento cutâneo, oral e de anexos cutâneos em paciente com Doença enxerto contra hospedeiro crônica expressa por inúmeras lesões de padrão liquenóide e de atrofia cutânea.

Dermoscopy of lichen aureus / Dermatoscopia do liquen aureus

Lichen aureus (also called "lichen purpuricus") is an uncommon subtype of pigmented purpuric dermatosis. Clinically characterized by rust macules, papules or plaques, it is a chronic disease which more often affects young adults and is localized mainly on the lower extremities. The diagnosis is made on the basis of clinical and histopathological features. Dermoscopy findings are useful to confirm clinical diagnosis.

O líquen aureus (também denominado "liquen purpuricus") é um subtipo pouco comum entre as dermatoses purpúricas pigmentadas. Clinicamente caracterizado por máculas, pápulas ou placas de coloração ferruginosa, é doença crônica, que acomete mais frequentemente adultos jovens e localiza-se principalmente nos membros inferiores. O diagnóstico pode ser feito a partir das características clínicas e histopatológicas, sendo os achados dermatoscópicos úteis para corroborar o diagnóstico clínico.

Queratosis liquenoide / Lichenoid keratosis

La queratosis liquenoide se presenta como una lesión solitaria, en forma de una mácula o pápula de color rojo, violáceo o marrón, que aparece predominantemente en mujeres y se localiza en el tronco. Su histología es semejante a la del liquen plano. Su origen es desconocido, aunque se presume que corresponde al resultado final de un proceso inflamatorio de una lesión preexistente, principalmente léntigo solar, queratosis actínica yqueratosis seborreica.

Lichenoid keratosis occurs as a solitary lesion in form of a red, purple or brown, macule or papule which appears predominantly in women, and in the trunk. Its histology is similar to lichen plannus. Its origin is unknown, although it is presumed that it corresponds to the final result of an inflammatory process of a preexistinglesion, mainly solar lentigo, actinic keratosis and seborrheic keratosis.

Reacción liquenoide en mucosa bucal asociada a restauración de amalgama: reporte de caso clínico / Lichenoid reaction in oral mucosa associated with amalgam restoration: case report

La reacción liquenoide en la mucosa bucal es una de las formas más frecuentes de las reacciones de hipersensibilidad retardada como respuesta inmune que puede estar relacionada al mercurio u otro componente de la amalgama. La reacción liquenoide es una lesión clínica e histológicamente indistinguible del liquen plano bucal. La única diferencia estriba en que desaparece al retirar el agente causante. Este trabajo tiene como objetivo presentar un caso de lesión liquenoide en mucosa bucal en una mujer de 34 años que solicito asistencia odontológica para diagnóstico y tratamiento de una lesión blanca en la mucosa yugal y encía vestibular derecha adyacente a los dientes 16 y 17 que presentaban restauración de amalgama. Se realizó una biopsia incisional y examen histopatológico teniendo un resultado compatible con el Liquen Plano. Con el consentimiento de la paciente las restauraciones de amalgama fueron substituidas por resina compuesta. Fue verificada la regresión continua de la lesión durante el seguimiento clínico del caso. El diagnóstico definitivo de lesiones liquenoides asociadas a la presencia de restauración de amalgama como factor etiológico es de suma relevancia porque de esta forma se obtiene un tratamiento basado en una intervención simplificada y local, diferente de la compleja actuación que ocurre en tratamientos que envuelven enfermedades auto-inmunes como el liquen plano.

Lichenoid reaction in the buccal mucosa is one of the most frequent forms of delayed hypersensitivity reaction as an immune response, which could be related to mercury or another amalgam component. It is a rare manifestation that is an important differential diagnosis in the group of buccal leucoplakias; and it is the reason of many controversies, particularly regarding lichen planus. The aim of this paper is to present a buccal mucosa lichenoid lesion case in a 34 year-old woman who requested odontologic assistance to be diagnosed and treated for a white lesion, located in the jugal mucosa and right vestibular gum, adjacent to teeth 16 and 17, which had amalgam restorations. An incisional biopsy and a histopatologic exam were made. The result was compatible with Liquen Planus. With the patient's consent the amalgam restorations were replaced with compound resin. A continuous regression of the lesion was verified during the clinical monitoring of the case. The final diagnosis of lichenoid lesions related to amalgam restorations as an etiologic factor is remarkable because by means of this a treatment based on a simplified and local intervention is obtained, which is different from the complicated procedure for treatments that involve auto-immune diseases like lichen planus.

Oral lichenoid lesions: Clinico-pathological mimicry and its diagnostic implications.

Oral cavity is commonly affected by number of lichenoid lesions, whose clinical and histopathologic features overlap due to the presence of inflammatory cells in connective tissue. Segregation of these lichenoid lesions is mandatory as each may embody a distinct disease entity in terms of cause, diagnosis and prognosis. The literature discussed in the article is an attempt to segregate individual lichenoid lesions by defining clinical and histopathologic variations among each other, which avoids the diagnostic problem.

Queratose liquenoide crônica: relato de caso / Keratosis lichenoides chronica: case report

A queratose liquenoide crônica ou doença de Nekam é uma dermatose mucocutânea rara da queratinização, com curso crônico e progressivo, que acomete geralmente indivíduos entre 20 e 40 anos. Existem, aproximadamente, 70 casos descritos na literatura. Devido à raridade desta dermatose e à ausência de tratamento efetivo, é uma doença de difícil manejo. No caso em questão, apresentamos um paciente de 42 anos com pápulas violáceas, hiperqueratósicas, algumas confluentes, com aspecto linear, rendilhado e em placas, localizadas no tronco e membros há cinco anos. Lesões aftoides na cavidade oral e úlceras rasas na genitália também faziam parte do quadro. O anatomopatológico foi bastante sugestivo de queratose liquenoide crônica. Introduziu-se tratamento com acitretina e dapsona, havendo melhora parcial do quadro.

Keratosis lichenoides chronica or Nekam's disease is a rare mucocutaneous dermatosis characterized by keratinization. It is chronic and progressive usually affecting individuals aged 20-40 years. Around 70 cases have been reported in the literature. Due to the rarity of this condition and the lack of effective treatment, it is a difficult disease to manage. In the case described below we present a 42-yearold patient with violaceous and hyperkeratotic papules in linear, reticular or plaque form, located on the trunk and limbs for five years. Aphthous lesions in the oral cavity and shallow ulcers on the genitalia also formed part of the clinical manifestation. Pathologic examination was suggestive of keratosis lichenoides chronica. Acitretin and dapsone was introduced and the lesions partially improved.

Líquen estriado no adulto / Lichen striatus on adult

Líquen estriado é uma erupção inflamatória incomum, de etiologia desconhecida. Raramente acomete adultos, e é caracterizada pelo surgimento abrupto de pápulas coalescentes, em arranjo linear, usualmente em extremidades. A histopatologia mostra reação liquenóide envolvendo folículos e glândulas. Ocasionalmente há sobreposição com líquen plano linear e "blaschkitis", seus principais diagnósticos diferenciais. Relata-se um caso de mulher adulta com pápulas eritematovioláceas em região cervical e hemiface direitas, cuja correlação clínico-histopatológica permitiu o diagnóstico de líquen estriado. Os achados atípicos e a dificuldade diagnóstica são discutidos.

Lichen Striatus is an uncommon inflammatory skin eruption of unknown etiology. It rarely affects adults, and it is characterized by abrupt onset of coalescent papules, in a linear disposition, usually on the extremities. Histopathology shows lichenoid reaction involving follicles and glands. Occasionally, there is overlap with linear lichen planus and "blaschkitis", the main differential diagnoses. It is reported here the case of an adult woman with erythematous violaceous papules on the right side of the neck and face, diagnosed with lichen striatus by clinical and histopathological correlation. The atypical findings and the diagnostic difficulty are discussed.

Queratosis liquenoide benigna / Benign lichenoid keratosis

La queratosis liquenoide benigna es una dermatosis relativamente frecuente, con presentación clínica en determinados ocasiones, que puede llegar o lo confusión con el melanoma. Se plantea que puede ser una lesión benigna en regresión. La dermatoscopia es de gran utilidad para lo orientación diagnóstica. El diagnóstico es histopatológico, por lo cual en los casos confusos lo realización de una biopsia está indicada. Se realizo uno revisión de lo literatura sobre la clínica, histopatología y dermatoscopia de lo queratosis liquenoide benigna.

Benign lichenoid keratosis is a relatively common dermatosis which clinical presentation in some cases causes confusion with melanoma. It is suggested that it may be a regressing benign lesion. Dermascopy is very useful for diagnostic orientation. The histopathological diagnosis, thus confusing where the biopsy is indicated. A review of the literature on benign lichenoid keratosis clinical, histopathology and dermascopy pattems is exposed.

Oral lichen planus versus oral lichenoid reaction: Difficulties in the diagnosis.

Lichen planus (LP) is a mucocutaneous disease with well-established clinical and microscopic features. The oral mucosa and skin may present clinical and microscopic alterations similar to those observed in LP, called lichenoid reactions (LRs), which are triggered by systemic or topical etiological agents. The difficulties faced to establish the differential diagnosis between the two pathologies were investigated in the literature. It was observed that the etiology of LP is still under discussion, with a tendency to self-immunity, while the etiology of LRs is related to the contact with specific agents, such as metallic restorative materials, resins, and drugs, allowing the establishment of a cause-effect relationship. In this case, the disease is caused by the antigen fixation in the epithelial cells, which are destructed by the immune system. Based on these data, protocols are suggested for this differentiation. The important role played by the integration between the clinician and the oral pathologist in the diagnostic process is highlighted. The treatment of LP comprises mainly the utilization of corticosteroids and the LR is treated by removal of the causal factor. Differentiation between the two diseases allows an effective and correct therapeutic approach.

Evaluation of mast cells in oral lichen planus and oral lichenoid reaction.

Recent attention has been directed towards the role of mast cells in the pathogenesis of oral lichen planus. Mast cells are responsible for trafficking of inflammatory cells into the connective tissue that in turn helps in progression and maintenance of the chronicity of oral lichen planus. In the present study we have evaluated the mast cell count and its cell types using toluidine blue stain, in oral lichen planus and compared it with oral lichenoid reactions. We have observed an increase in mast cell count in both these conditions in comparison to normal mucosa. Also, a significant difference in the types of mast cells in oral lichen planus and oral lichenoid reactions has been noted, possibly suggesting that the role of mast cell is different in these conditions.

Liquenificación gigante de Pautrier ulcerada / Ulcerated giant lichenification of Pautrier

La liquenificación gigante de Pautrier es una forma rara y exhuberente de liquen simple crónico que resulta del rascado intenso de zonas intertriginosas tales como genitales, ingles y axilas. Presentamos un caso de esta rara afección en el que las lesiones aparecían ulceradas haciendo de esta manera difícil el diagnóstico

Erupción liquenoide por reveladores a color: a propósito de un caso / Lichenoid eruption by color developers: a purpose of a case

Las erupciones liquenoides son un grupo de dermatosis con un cuadro semejante y en ocasiones indistinguible desde un punto de vista clínico e histológico al liquen plano. Se reporta el caso de un paciente masculino, de 46 años de edad, de ocupación fotógrafo con diagnóstico de erupción liquenoide por reveladores a color, enfermedad poco frecuente que se manifiesta con lesiones similares al liquen plano localizadas en zonas expuestas al producto industrial y se realiza revisión de la literatura

Líquenes planos sistematizados: a propósito de 10 casos / Systematized lichen planus: review on 10 cases

Presentamos 10 casos de líquenes planos sistematizados de los cuales 8 eran mujeres con un promedio de edad de 37 años, localizados predominantemente en tórax y miembros superiores y una forma hemilingual no descrita en la literatura. Ocho de ellos parecían disponerse siguiendo metámeras, excepto dos que adoptaban un patrón según se describen las líneas de Blaschko. Seis refirieron prurito de leve a moderado