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.

Different distribution patterns of plasmacytoid dendritic cells in discoid lupus erythematosus and lichen planopilaris demonstrated by CD123 immunostaining

Abstract Background: Clinical and histological features may overlap between lichen planopilaris-associated and discoid lupus erythematosus-associated scarring alopecia. Objectives: The aim of this study was to demonstrate the cutaneous infiltration of plasmacytoid dendritic cells and to compare their distribution pattern in discoid lupus erythematosus and lichen planopilaris. Methods: Twenty-four cases of discoid lupus erythematosus and 30 cases of lichen planopilaris were examined for immunostaining of the CD123 marker. The percentage and distribution pattern of plasmacytoid dendritic cells and the presence of the plasmacytoid dendritic cells clusters were evaluted in the samples. Results: The number of plasmacytoid dendritic cells was higher in the discoid lupus erythematosus specimens. Aggregations of 10 cells or more (large cluster) were observed in half of the discoid lupus erythematosus specimens and only 2 lichen planopilaris, with 50% sensitivity and 93% specificity for differentiating discoid lupus erythematosus from lichen planopilaris. Study limitations: Incidence and prevalence of discoid lupus erythematosus-associated scarring alopecia in the scalp are low, so the samples size of our study was small. Conclusions: We suggest that a plasmacytoid dendritic cells cluster of 10 cells or more is highly specific for distinguishing discoid lupus erythematosus from lichen planopilaris. It also appears that CD123 immunolabeling is valuable in both active and late stages of the disease.

Brimonidine-induced unilateral ocular lichen planus: a case report / Líquen plano ocular unilateral induzido por brimonidina: relato de caso

ABSTRACT This report was written to describe a case of unilateral brimonidine-induced conjunctival lichen planus. Because the ophthalmic examination indicated chronic conjunctivitis or drug-induced pseudopemphigoid, the patient underwent thorough ophthalmic and systemic examinations, as well as conjunctival biopsy and direct immunofluorescence studies. A 71-year-old woman with unilateral left eye findings of chronic conjunctivitis was referred to our Ophthalmology Department. The patient reported that chronic conjunctivitis began shortly after she initiated use of topical brimonidine. Ophthalmic examination revealed foreshortening of the inferior fornix and symblepharon. Conjunctival biopsy revealed submucous lymphocytes and shaggy distribution of fibrinogen on direct immunofluorescence; this was suggestive of ocular lichen planus. No other systemic lesions were found that were consistent with the presentation of lichen planus. A good response was observed to topical cyclosporine treatment. To our knowledge, this may be the first report of unilateral ocular lichen planus without systemic findings. The correlation with the initiation of topical brimonidine suggests that this might be the first case of biopsy-confirmed brimonidine-induced ocular lichen planus.

RESUMO Este relato é para descrever um caso de líquen plano conjuntival unilateral induzido por brimonidina. Como o exame oftalmológico indicava conjuntivite crônica ou pseudopenfigóide induzido por medicamento, o paciente foi submetido a exames oftalmológicos e sistémicos completos, além de biópsia conjuntival e estudos de imunofluorescência direta. Uma mulher de 71 anos de idade com achados unilaterais do olho esquerdo de conjuntivite crônica foi encaminhada ao nosso departamento de Oftalmologia. A paciente relatou que a conjuntivite crônica começou logo após o início do uso da brimonidina tópica. O exame oftalmológico revelou encurtamento do fórnice inferior e do symblepharon. A biópsia conjuntival revelou linfócitos submucosos e distribuição felpuda de fibrinogênio na imunofluorescência direta; isso era sugestivo de líquen plano ocular. Não foram encontradas outras lesões sistêmicas compatíveis com a apresentação do líquen plano. Uma boa resposta foi observada no tratamento tópico com ciclosporina. Pelo nosso conhecimento, este pode ser o primeiro relato de líquen plano ocular unilateral sem achados sistêmicos. A correlação com o início da brimonidina tópica sugere que este pode ser o primeiro caso de líquen plano ocular induzido por brimonidina confirmado por biópsia.

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.

Esophageal lichen planus: An unusual cause of dysphagia in the elderly / Líquen plano esofágico: uma causa incomum de disfagia em idosos

Summary An 82-year-old man sought our service with dysphagia and was referred for upper endoscopy with biopsies, which evidenced multiple ulcers of the esophagus and oropharinx. Histopathology confirmed the unusual diagnosis of esophageal lichen planus. The correct clinical suspicion of this disease can facilitate the diagnosis and guide specific treatment, which can drastically change the natural course of the disease.

Resumo Paciente do sexo masculino, de 82 anos, com disfagia, foi encaminhado para realização de endoscopia digestiva alta com biópsias, na qual foram evidenciadas múltiplas úlceras de esôfago e orofaringe. O estudo histopatológico confirmou o diagnóstico raro de líquen plano esofágico. A correta suspeita clínica dessa doença pode facilitar o diagnóstico e direcionar para um tratamento específico, o que pode drasticamente alterar o curso natural dessa comorbidade.

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.

Piccardi-Lassueur-Graham-Little syndrome associated with frontal fibrosing alopecia

Abstract: Piccardi-Lassueur-Graham-Little syndrome is a rare entity characterized by progressive scarring alopecia of the scalp and keratotic papules on hairless skin, associated with non-scarring alopecia in the axilla and pubic area or lichen planus lesions. We describe the case of a 70-year-old woman who presented a Piccardi-Lasseur-Graham-Little syndrome, along with frontal fibrosing alopecia.

Lichen planopilaris developed during childhood

Abstract: Lichen planopilaris is a disease that appears with lymphocytic cicatricial alopecia. It is considered a follicular variant of lichen planus. The examination of affected areas shows alopecia with perifollicular erythema and scaling, revealing a predilection for hair follicles. The involvement of children is uncommon, with few reports in this population in the literature. This study presents a clinical case of a male patient of 15 years of age with characteristic lesions of lichen planopilaris.

Frontal fibrosing alopecia and extrafacial lichen planus pigmentosum in a caucasian woman

Abstract We present the case of a 72-year-old Caucasian woman with frontal fibrosing alopecia of one year's duration. Eighteen months later, she presented with sudden hyperpigmentation on the submental area, neck and upper chest. Histopathological examination was consistent with lichen planus pigmentosus. This is the report of a unique case of a FFA and extrafacial lichen planus pigmentosus.

Frontal fibrosing alopecia and lichen planus pigmentosus: diagnosis and therapeutic challenge

Abstract Frontal fibrosing alopecia is a variant of lichen planopilaris with marginal progressive hair loss on the scalp, eyebrows and axillae. We report a case of frontal fibrosing alopecia and lichen planus pigmentosus in a postmenopausal woman, that started with alopecia on the eyebrows and then on the frontoparietal region, with periocular and cervical hyperpigmentation of difficult management. The condition was controlled with systemic corticosteroid therapy and finasteride. Lichen planus pigmentosus is an uncommon variant of lichen planus frequently associated with frontal fibrosing alopecia in darker phototipes. It should be considered in patients affected by scarring alopecia with a pattern of lichen planopilaris and areas of skin hyperpigmentation revealing perifollicular hyperpigmentation refractory to multiple treatments. This case illustrates diagnostic and therapeutic challenge in face of scarring alopecia and perifollicular hyperpigmentation.

"String of pearls pattern": report of three cases of non clear-cell acanthoma

Abstract The coiled and dotted vessels in a serpiginous arrangement or "string of pearls" is considered a classical vascular pattern associated with clear cell acanthoma. We present three cases of epidermal tumors different from clear cell acanthoma that have the same "string of pearls" vascular pattern. Even though most authors keep considering the "string of pearls" vascular pattern an almost pathognomonic sign of clear-cell acanthoma, the cases presented here suggest that some other epidermal tumors can also show this pattern.

DHEA and frontal fibrosing alopecia: molecular and physiopathological mechanisms

Abstract The transforming growth factor-beta 1 (TGFβ1) promotes fibrosis, differentiating epithelial cells and quiescent fibroblasts into myofibroblasts and increasing expression of extracellular matrix. Recent investigations have shown that PPAR (peroxisome proliferator-activated receptor*) is a negative regulator of fibrotic events induced by TGFβ1. Dehydroepiandrosterone (DHEA) is an immunomodulatory hormone essential for PPAR functions, and is reduced in some processes characterized by fibrosis. Although scarring alopecia characteristically develops in the female biological period in which occurs decreased production of DHEA, there are no data in the literature relating its reduction to fibrogenic process of this condition. This article aims to review the fibrogenic activity of TGFβ1, its control by PPAR and its relation with DHEA in the frontal fibrosing alopecia.

Lichen planopilaris-like eruption during treatment with tyrosine kinase inhibitor nilotinib

Abstract Tyrosine kinase inhibitors are effective as a target therapy for malignant neoplasms. Imatinib was the first tyrosine kinase inhibitor used. After its introduction, several other drugs have appeared with a similar mechanism of action, but less prone to causing resistance. Even though these drugs are selective, their toxicity does not exclusively target cancer cells, and skin toxicity is the most common non-hematologic adverse effect. We report an eruption similar to lichen planopilaris that developed during therapy with nilotinib, a second generation tyrosine kinase inhibitor, in a patient with chronic myeloid leukemia resistant to imatinib. In a literature review, we found only one report of non-scarring alopecia due to the use of nilotinib.

Frontal fibrosing alopecia in association with Sjögren's syndrome: more than a simple coincidence

Abstract Frontal fibrosing alopecia is a distinctive form of scarring alopecia considered to be a clinical variant of lichen planopilaris. It predominantly occurs in postmenopausal women and has a slowly progressive course. It was first described by Kossard in 1994. Since then the number of reported cases has increased significantly. Coexistence of frontal fibrosing alopecia and autoimmune disorders - such as discoid erythematosus lupus and Sjögren's syndrome - may suggest a common pathogenic background among the diseases.

Coexistence of nail lichen planus and lichen planus pigmentosus

Abstract We describe a fifty-six-year old, Afro-descendent female patient showing dystrophy of her twenty nails and hyperchromic, asymptomatic macule on her face. Histopathological examination of the macule showed vacuolization of the basal layer, melanophages in the superficial dermis and lymphoplasmocytic inflammatory infiltrate. Nail biopsy revealed orthokeratotic hyperkeratosis and lichenoid inflammatory infiltrate. Lichen planus pigmentosus is an uncommon variety of lichen planus. It is characterized by typical hyperpigmented macules on the face and neck. Nail changes might be present in 10% of lichen planus cases, but no associations with lichen planus pigmentosus have been described. We report a case of lichen planus in twenty nails associated with lichen planus pigmentosus on the patient's face.