Liquen plano pigmentoso en la cavidad oral / Lichen planus pigmentosus in oral cavity

Introducción: El liquen plano pigmentoso es una lesión autoinmune de etiología desconocida, aunque tiene mayor incidencia en mujeres de mediana edad, a las que afecta principalmente la cara y el cuello y, en menor medida, la cavidad oral. Objetivo: Describir las características clínicas e histopatológicas de un caso de liquen plano pigmentoso en la cavidad oral. Presentación del caso: Mujer de 21 años, negra, que acudió al servicio quejándose de una mancha en la cavidad oral. Las lesiones presentaban un mes de evolución, crecimiento radial y sin síntomas dolorosos. Consistían en placas ennegrecidas de contorno regular con estrías blanquecinas en su periferia, forma redondeada, bordes afilados, en mucosa yugal bilateral, que medían aproximadamente 25 mm en el derecho y 13 mm en el lado izquierdo. Después de la biopsia incisional y el análisis histopatológico, se confirmó la sospecha de liquen plano pigmentoso oral. El tratamiento propuesto para las lesiones fue conservador mediante un estricto seguimiento clínico. Conclusiones: El diagnóstico del liquen plano pigmentoso, debido a su baja ocurrencia en la cavidad oral y sus características clínicas atípicas y semejantes a otras lesiones orales, es complejo. En este contexto, se ratifica la relevancia del examen histopatológico y se destaca la necesidad de otros estudios para aclarar los factores etiológicos involucrados en esta lesión(AU)

Introduction: Pigment lichen planus is an autoimmune lesion of unknown etiology, with preference for middle-aged women, which mainly affects the face and neck, being rare in the oral cavity. Objective: To report a case of pigment lichen planus in the oral cavity, with emphasis on its clinical and histopathological characteristics. Case report: 21 years old woman, black, who came to the service complaining about a spot in the oral cavity. The lesions presented a month of evolution, radial growth and no painful symptoms. They consisted of blackened plates of regular contour with whitish stretch marks on their periphery, rounded shape, sharp edges, on bilateral jugular mucosa, which measured approximately 13 mm on the left side and 25 mm on the right. After the incisional biopsy and histopathological analysis, the suspicion of oral pigment lichen planus was confirmed. The proposed treatment for the lesions was conservative through strict clinical follow-up. Conclusion: The importance and difficulty of the diagnosis of pigment lichen planus is emphasized, especially due to its low occurrence in the oral cavity and its atypical clinical characteristics and similar to other oral lesions. In this context, the relevance of the histopathological examination is ratified and the need for further studies to clarify the etiological factors involved in this pathology is highlighted(AU)

Liquen plano, estudio retrospectivo en un hospital pediátrico / Lichen planus, retrospective study in a pediatric hospital

Introducción: El liquen plano (LP) es una enfermedad inflamatoria crónica, de etiología desconocida, que puede afectar la piel, los anexos y las mucosas. Objetivo: Describir las características epidemiológicas y clínicas y el tratamiento realizado en los pacientes con diagnóstico clínico e histopatológico de LP, valorados en el Servicio de Dermatología del Hospital de Pediatría "Prof. Dr. Juan P. Garrahan" en los últimos 33 años. Diseño:Estudio descriptivo, retrospectivo y transversal. Materiales y métodos: Se revisaron las historias clínicas de los pacientes con diagnóstico histopatológico de LP atendidos en el Hospital desde agosto de 1987 hasta febrero de 2020 y se analizaron las variables epidemiológicas, clínicas y de tratamiento. Resultados: Se incluyeron datos de 26 pacientes, 18 de los cuales eran varones (69,3%). La media de edad en el momento del diagnóstico fue de 9,6 años. Presentaron la variedad de LP clásico 24 pacienes (93%). El compromiso ungueal y de la mucosa bucal se observó en 2 pacientes (7,7%). Ninguno tuvo asociación a fármacos ni a infecciones. En cuanto al tratamiento recibido, 4 pacientes (15,4%) tuvieron pérdida del seguimiento; 13 (50%) realizaron tratamiento tópico; 6 (23%), tratamiento sistémico y 3 (11,5%), tratamiento combinado. El promedio de seguimiento fue de 6 meses y se obtuvo una respuesta favorable en el 68% de los pacientes. Conclusiones: El LP es una patología de baja frecuencia en la infancia. No se encontró asociación a medicamentos ni a enfermedades infecciosas como se describe en la población adulta. Su curso es crónico y, en los pacientes que tuvieron un adecuado seguimiento, se obtuvo una buena respuesta al tratamiento indicado.

Introduction: Lichen planus (LP) is a chronic, inflammatory disease of unknown etiology, which can affect skin, adnexal tissues and mucosa. Objective: to describe the clinical, epidemiological characteristics and the treatment received by patients with clinical and histopathological diagnosis of LP, seen at the Dermatology Service of Pediatric Hospital "Prof. Dr. Juan P. Garrahan" in the last 33 years. Design: Descriptive, retrospective and cross-sectional study. Materials y methods: We reviewed the clinical history of patients with histopathological diagnosis of LP that were treated at our hospital from August 1987 to February 2020 and analysed the epidemiological, clinical and treatment variables. Results: Data of 26 patients were included in this study 18 of which are males (69.3%). The mean age of diagnosis was 9.6 years. 24 patients (93%) presented the classical type of LP. Ungual and mucosal compromise were observed in 2 patients (7.7%) and none had associations with infections or drugs. Regarding treatment, 4 patients (15.4%) did not continue follow-ups, 13 patients (50%) had topical treatment, 6 (23%) had systemic treatment and 3 patients (11.5%) received combined treatment. The average follow-up time was 6 months and 68% of patients had favorable outcome. Conclusions: LP is an uncommon pathology in children. During this study we did not find any etiologic relations with use of drugs or infectious diseases as is described in de adult population. Its course is chronic and in the patients that had a proper follow-up a good response to the indicated treatment was obtained.

Lichen planus with multiple system involvement including the mouth, vagina, urethra, and scalp: a case report

This is a case report of lichen planus (LP) with multiple system involvement. A 35-year-old female patient was admitted in November 2014 with a 5-year history of painful/difficult sexual intercourse and loss of oral mucosa, and an 8-year history of focal hair loss. Earlier, the patient had been unable to adhere to corticosteroid therapy because of severe adverse side effects. In September 2014, labia minora mucosa defects and stricture of the urethral orifice (with dysuria), vaginal orifice, and vagina were identified. Biopsy was performed and a diagnosis of erosive LP was made. The patient was treated with an oral immunosuppressant (cyclosporine A) and urethral/vaginal dilatation. Urine flow rate and sex life were improved after 6 months and she discontinued medication. Four years later, the patient reported a good overall treatment efficacy. LP can involve multiple systems and should be considered in patients with dyspareunia. Immunosuppressive agents can achieve a satisfactory effect in patients with contraindication to corticosteroid.

Liquen plano blaschkoide facial como expresión de mosaicismo cutáneo, a propósito de un caso / Lichen planus blaschkoide facial as expression of cutaneous mosaicism, on a case by case

El liquen plano es un trastorno inflamatorio adquirido de etiología desconocida que, excepcionalmente, puede presentarse de forma lineal, debido a la predisposición genética de un clon que se produce durante el desarrollo embrionario. El liquen plano lineal o Blaschkoide de localización facial, es aún más infrecuente, y traduce una mutación genética postcigótica, que así como en otras patologías inflamatorias dermatológicas, aumenta la susceptibilidad de los individuos a desarrollarla.

Lichen planus is an acquired inflammatory disorder of unknown etiology that in exceptional cases can occur linearly. This is due to the genetic predisposition of a clone that occurs during embryonic development. Facial localization of the lichen planus is even more infrequent, and translates to a postcigotic genetic mutation. This mutation increases individual susceptibility, just as in other dermatological inflammatory pathologies.

Bilateral retinal vasculitis in a patient with lichen planus / Vasculite retiniana bilateral em um paciente com líquen plano

ABSTRACT Lichen planus (LP) is an autoimmune, inflammatory disease of unknown etiology that commonly affects the skin and mucous membranes. Retinal vasculitis is a group of vision-threatening disorders, in which autoimmunity is thought to play a role in pathogenesis. We present the case of a patient who was diagnosed with retinal vasculitis and who was followed up for mucosal LP. LP has not been reported as a cause of retinal vasculitis in the literature. We believe that the retinal vasculitis in this case was related to LP because cellular immunity plays a role in the pathogenesis of both entities.

RESUMO Líquen plano (LP) é uma doença auto-imune, inflamatória de etiologia desconhecida que normalmente afeta a pele e membranas mucosas. Vasculite retiniana engloba um grupo de doenças que ameaçam a visão em que a autoimunidade parece desempenhar um papel na sua patogênese. Apresentamos um caso que foi diagnosticado com vasculite da retina e que tinha sido acompanhado com LP de mucosa. LP não consta entre as causas de vasculite retiniana na literatura. Acreditamos que a vasculite da retina no nosso caso, foi relacionado com o LP considerando que a imunidade celular desempenha um papel na patogênese de ambas as entidades.

Dermatosis cenicienta: revisión y diagnóstico diferencial con otros trastornos pigmentarios idiopáticos / Ashy dermatosis: revision and differential diagnosis with other idiopathic pigmentary disorders

La dermatosis cenicienta es un trastorno pigmentario infrecuente que se presenta en individuos de piel morena, especialmente en centroamericanos. Las lesiones consisten en manchas hiperpigmentadas de coloración gris-azulada, principalmente localizadas en la cara, tronco y brazos. Su principal diagnóstico diferencial debe ser planteado con tres dermatosis pigmentarias de origen idiopático: eritema discrómico perstans, pigmentación macular eruptiva idiopática y liquen plano pigmentoso. Hay consenso en considerar al eritema discrómico perstans como la misma enfermedad, pero con un borde eritematoso transitorio. La pigmentación macular eruptiva idiopática es clínicamente similar, pero sin una dermatitis de interfase al estudio histopatológico, y sólo muestra una pigmentación de la capa basal, incontinencia pigmentaria y melanófagos en la dermis. Finalmente, el liquen plano pigmentoso debe ser considerado como una variedad pigmentada de liquen plano.

Ashy dermatosis is an infrequent disorder of pigmentation, which predominantly occurs in darkly pigmented individuals, specially in central americans. The lesions consist of bluish-gray hyperpigmented patches mainly located on the face, trunk and arms. Its main differential diagnosis must be raised with three idiopathic pigmentary disorders: erythema dyschromicum perstans, idiopathic eruptive macular pigmentation and lichen planus pigmentosus. It has been widely accepted that erythema dischromicum perstans is the same disease, but with a transient erythematous border. Idiopathic eruptive macular pigmentation is clinically similar but without an interfase dermatitis at histopathologic studies, only showing increased pigmentation of the basal layer and pigmentary incontinence and many melanophages in the dermis. Finally, lichen planus pigmentosus must be considered as a pigmented variant of lichen planus.

Síndrome Graham-Little-Piccardi-Lasseur: a propósito de un caso y revisión de la literatura / Graham-Little-Piccardi-Lasseur syndrome: about a case and review of the literature

El Síndrome Graham-Little-Piccardi-Lasseur (GLPLS) corresponde a una variante clínica del llamado Liquen plano pilar y se caracteriza por presentar alopecia cicatrizal progresiva de cuero cabelludo con las características del Liquen plano pilar, alopecia no cicatrizal que compromete axilas y pubis, y erupción folicular liquenoide en tronco, extremidades, cara y/o cejas. Presentamos el caso de una paciente de 19 años que ha sido diagnosticada con GLPLS y cuyo curso ha sido de difícil manejo.

The Graham-Little-Piccardi-Lasseur Syndrome (GLPLS) corresponds to a clinical variant of Lichen planopilaris and is characterized by progressive scarring scalp alopecia with features of flat lichen, non-scarring alopecia localized in the armpits and pubis, and lichenoid follicular eruption in trunk, extremities, face and / or eyebrows. We present the case of a 19-year-old female patient who has been diagnosed with GLPLS and whose course has been difficult to manage.

Frontal fibrosing alopecia and lichen planopilaris in HLA-identical mother and daughter.

Frontal fi brosing alopecia (FFA) is a lymphocyte-mediated scarring alopecia thought to be a variant of lichen planopilaris (LPP). We present a 67-year-old woman with frontal fi brosing alopecia whose daughter was diagnosed to have lichen planopilaris. Both patients had identical human leukocyte antigen (HLA) D types, supporting a phenotypical relationship between the two clinical entities. Interestingly, our patient also had of autoimmune chronic atrophic gastritis, a previously unreported association.

Estudio retrospectivo de pacientes con alopecia fibrosante frontal / Retrospective study of patients with frontal fibrosing alopecia

Introducción: La alopecia fibrosante frontal, considerada por algunos autores como un subtipo de liquen plano pilaris, corresponde a una alopecia cicatricial primaria, que se presenta en mujeres adultas y se caracteriza por el retroceso simétrico de la línea de implantación fronto temporal. Se presenta un estudio descriptivo de pacientes con Alopecía fibrosante frontal atendidos en este centro. Objetivos: Evaluar características clínicas, dermatoscópicas e histopatológicas de pacientes con alopecia fibrosante frontal. Materiales y métodos: Estudio retrospectivo de pacientes con diagnóstico clínico-histopatológico de alopecia fibrosante frontal atendidos en nuestro centro desde Julio 2010 a Octubre 2012. Resultados: Se encontraron 57 casos con diagnóstico histológico de alopecia cicatricial, de los cuales 8 (14 por ciento) se diagnosticaron como Alopecia Fibrosante Frontal. El 100 por ciento correspondieron a mujeres, el promedio de edad fue de 45 años. Sólo la mitad de los casos comenzó con la alopecia luego del climaterio. El hallazgo clínico más frecuentemente encontrado fue el retroceso de la línea de implantación frontotemporal (87,5 por ciento); y en segundo lugar la disminución difusa de densidad capilar (37,5 por ciento) y alopecia de la cola de las cejas (50 por ciento). No se reportó ningún caso asociado a liquen plano cutáneo o de mucosas. Los hallazgos más relevantes a la dermatoscopía fueron: eritema perifolicular (50 por ciento) e hiperqueratosis folicular (25 por ciento). A la histopatología, los principales hallazgos fueron el infiltrado inflamatorio linfocitario perifolicular (50 por ciento) y fibrosis concéntrica perifolicular (100 por ciento). Discusión: La Alopecia fibrosante frontalrepresenta el 14 por ciento de las alopecias cicatriciales primarias en nuestra serie. La edad al diagnóstico fue inferior a lo reportado en la literatura. El principal hallazgo clínico fue el retroceso de la línea de implantación frontotemporal...

Introduction: Frontal fibrosing alopecia, considered by some authors as a subtype of lichen planopilaris, is a scarring alopecia that usually involves adult women and is characterized by the symmetric recession of fronto-temporal hairline. Objectives: Characterize the clinical, dermoscopic and histological features of frontal fibrosing alopecia. Materials and Methods: We conducted a retrospective study of patients with clinical and histological diagnosis of Frontal Fibrosing Alopecia treated in our institution from July 2010 to October2012. Results: 8 out of 57 cases with histological diagnosis of scarring alopecia, had diagnosis of frontal fibrosing alopecia (14 percent). 100 percent were women, with mean age of 45 years. 50 percent of patients began with alopecia in the post-climacteric period. Recession of frontotemporal hair-line was the most common finding (87.5 percent), associated with reduced capillary density (37.5 percent) and loss of eyebrows (50 percent). In our study, none of the patients had other signs of lichen planus. At dermoscopy, most common finding were perifollicular erythema (50 percent) and follicular hyperkeratosis (25 percent). Most common histological findings were a perifollicular lymphocytic inflammatory infiltrate (50 percent) and perifollicular concentric fibrosis (100 percent). Discussion: Frontal fibrosing alopecia represents 14 percent of scarring alopecia in our series. Age at diagnosis was lower than reported in the literature, and only 50 percent of women presented alopecia in the postmenopausal period. The main clinical finding was the recession of frontotemporal hairline...

Association of alopecia areata with segmental vitiligo and segmental lichen planus: a case report

Alopecia areata [AA] is a chronic inflammatory condition that affects the hair follicles and sometimes, the nails. It is believed that AA is caused by T cell-mediated autoimmune response and increased frequency of other autoimmune diseases in persons suffering from AA supports this hypothesis. A 12-year-old boy presented with extensive alopecia areata with pitting in all nails for 6 years. He had developed lesions of vitiligo and lichen planus on trunk in a segmental manner. While the association of alopecia areata and vitiligo as well as lichen planus is well known, segmental nature of both vitiligo and lichen planus is quite rare

Perda pilosa por líquen plano pilar após transplante capilar: relato de dois casos e revisão da literatura / Hair loss due to lichen planopilaris after hair transplantation: a report of two cases and a literature review

Alopecia androgenética é tratada com frequência por meio de microtransplante capilar, téc nica em que os fios transplantados geralmente não caem, pois mantêm características da área doadora, mais resistente. O líquen plano pilar é uma alopecia cicatricial com permanente destruição pilosa. Este artigo relata dois casos de lesões compatíveis com líquen plano pilar em áreas receptora e doadora póstransplante. A dominância da área doadora foi aparentemente sobrepujada pelo líquen plano pilar, que deve ter gerado a queda dos fios. Relatos semelhantes são raros. À suspeita de líquen plano pilar, devese biopsiar o couro cabeludo e evitar o transplante durante a atividade da doença.

Androgenetic alopecia is often treated by follicular unit transplantation, a technique that involves minimal risk of hair loss because of the more resistant nature of the donor area. Lichen planopilaris is a cicatricial alopecia that causes permanent destruction of hair follicles. We report two cases of post-transplantation lesions compatible with lichen planopilaris in both recipient and donor areas. The quality of the hair follicles in the donor area was apparently compromised by lichen planopilaris, the probable cause of hair loss. Similar reports are rare. When lichen planopilaris is suspected, a biopsy of the scalp must be performed to avoid transplantation during disease activity.

Liquen plano / Lichen planus

El liquen plano (LP) es una dermatosis inflamatoria crónica que afecta a la piel, membranas mucosas, pelo y uñas; de etiología desconocida, en cuya patogenia existe un daño autoinmunitario mediado por linfocitos T frente a los queratinocitos basales que expresan autoantígenos alterados en su superficie. Se presenta con diferentes patrones clínicos, como LP oral, LP anular, LP lineal, LP atrófico, LP hipertrófico, LP ampollar, LP pigmentoso, LP actínico. Su presentación clásica se caracteriza por pápulas poligonales o placas, pruriginosas, de coloración violácea, superficie brillante, surcada por estrias blanquecinas, característicamente distribuidas en forma simétrica sobre todo en las superficies flexoras de la muñeca, tobillos y muslos. Evoluciona por brotes y su curso es autolimitado, se resuelve en un periodo de meses a años, pero puede persistir indefinidamente. Existen múltiples opciones de tratamiento que incluyen corticoides tópicos, intralesionales y sistémicos, retinoides, fototerapia, inmunomoduladores tópicos y en casos graves o resistentes al tratamiento, la ciclosporina, metotrexato y fotoféresis extracorpórea.

Lichen planus (LP) is a chronic inflammatory dermatosis that affectsthe skin, mucous membranes, hair and nails; of unknown aetiology, whose pathogenesis is autoimmune damage mediated by T cells against basal keratinocytes that express autoantigens altered on its surface. It comes with different clinical patterns, such as oralLP, LP annularis, LP linearis, LP, LP verrucosus, bullosa LP, LP pigmentosus, LP actinicus. Classical presentation is characterized bypolygonal papules or plaques, pruritic, violet colour, glossy surface, drained by whitish stretch marks, characteristically distributed in symmetrical form especially in surface flexors of the wrist, ankles and thighs. It evolves by outbreaks and its course is self-limited, resolving in a period of months to years, but may persist indefinitely. There are multiple treatment options including steroids topics, miscellaneous and systemic, retinoids, phototherapy, immunomodulators topical and in cases of severe or resistant to treatment, ciclosporin, methotrexate and photoferesis extracorporea.

Liquen plano pigmentoso / Lichen planus pigmentosus

El liquen plano pigmentoso (LPP) es una variante relativamente rara de liquen plano (LP), se define como un desorden crónico pigmentario caracterizado por máculas hiperpigmentadas que afecta áreas expuestas al sol como la cara, cuello y extremidades superiores. Presentamos el caso de una paciente mujer de 23 años con una dermatosis pigmentaria anular en rostro que resultó clínica e histopatológicamente compatible con liquen plano pigmentoso. Se reporta el caso por la poca frecuencia de esta patología, la misma que debe entrar en el diagnóstico diferencial de las dermatosis hiperpigmentadas en zonas fotoexpuestas.

The lichen planus pigmentosus (LPP) is arelatively rare variant of flat lichen (LP), isdefined as a chronic pigmentary disorder characterized by hyperpigmented stains that affects areas exposed to the Sun as the face, neck and upper extremities. LetÆs sense beforehand the case of a patient woman of 23 years old with a pigmentary dermatosis to annul in face that turned out to be clinical and histopatológicamente compatiblly with flat lichen pigmentoso. The case brings for small frequency of this pathology, the same one that must enter the differential diagnosis of the hyperpigmented dermatosis photoexposed zones.