Determinação da atividade antitirosinase e antioxidante de Myrsine coriacea (Sw. ) R. Br. ex Roem. & Schult. (Primulaceae) / Determination of the antityrosinase and antioxidant activity of Myrsine coriacea (Sw. ) R. Br. ex Roem. & Schult. (Primulaceae)

myrsine coriacea (Sw.) R. Br. ex Roem. & Schult. (Primulaceae) conhecida popularmente como capororoquinha ou capororoca, é amplamente distribuída nas regiões sul e sudeste do Brasil. As espécies desse gênero apresentam um potencial antioxidante e anti-inflamatório, que pode ser acessado na busca de novos ativos para o tratamento de desordens pigmentares da pele. Desta forma, este trabalho teve como objetivos avaliar o potencial antitirosinase e antioxidante de extratos e frações de M. coriacea e identificar os possíveis compostos responsáveis por essas atividades. Foram realizados ensaios para avaliar o potencial antioxidante das amostras através do método do DPPH, enquanto a capacidade hipopigmentante das amostras foi avaliado pela inibição da enzima tirosinase. Como complemento, foram determinados os teores de compostos fenólicos totais e flavonoides através dos métodos colorimétricos empregando o reagente Folin-Ciocalteau e AlCl3. Adicionalmente, os extratos de M. coriacea tiveram avaliados seus potenciais citotóxicos utilizando diferentes linhagens tumorais humanas. O perfil fitoquímico de M. coriacea foi analisado por cromatografia a gás acoplada com espectrometria de massas (CG-EM) e cromatografia em camada delgada (CCD) com padrões. Nessas análises foram identificados 34 compostos, sendo o ácido palmítico e o palmitato de etila os compostos majoritários nas amostras de M. coriacea. O extrato bruto das folhas apresentou o maior teor de fenólicos totais, enquanto a fração de acetato de etila das folhas teve o maior teor de flavonoides. Contudo, o extrato bruto dos frutos apresentou a melhor atividade antioxidante de todas as amostras analisadas, apresentando também a melhor atividade antitirosinase. Dentre os compostos anotados, mandenol, ácido -linoleico e o linolenato de etila foram os compostos considerados como possíveis inibidores da tirosinase, com boa interação molecular com a enzima nas análises de ancoragem molecular in silico. Das amostras analisadas com relação a inibição de crescimento frente as células tumorais, a amostra da fração de clorofórmio das folhas foi a que apresentou potencial antitumoral frente as células de adenocarcinoma de cólon (HCT116)

myrsine coriacea (Sw.) R. Br. ex Roem. & Schult. (Primulaceae) popularly known as capororoquinha or capororoca, is widely distributed in southern and southeastern Brazil. Myrsine species have an antioxidant and anti-inflammatory potential, which can be accessed in the search for new actives for the treatment of skin pigmentation disorders. Thus, this work aimed to evaluate the antityrosinase and antioxidant potential from extracts and fractions of M. coriacea and to identify the probable compounds responsible for these activities. Assays were performed to evaluate the antioxidant potential of the samples using the DPPH method, while the hypopigmentation capacity of the samples was evaluated by the tyrosinase inhibition. As a complement, the amounts of total phenolic compounds and flavonoids were determined through colorimetric methods using the Folin-Ciocalteau reagent and AlCl3. Additionally, M. coriacea extracts had their cytotoxic potential evaluated using different human tumor cell lines. M. coriacea phytochemical profile was obtained by gas chromatography coupled with mass spectrometry (GC-MS) and thin layer chromatography (TLC) with standards. In these analyses, 34 compounds were identified, with palmitic acid and ethyl palmitate as the major compounds in M. coriacea samples. The leaf crude extract presented the highest total phenolics contents, while the leaf ethyl acetate fraction had the highest flavonoid amounts. However, the fruit crude extract showed the best antioxidant and antityrosinase activities of all analyzed samples. Among the annotated compounds, mandenol, -linoleic acid and ethyl linolenate were the compounds considered as putative tyrosinase inhibitors, presenting good molecular interaction with the enzyme active site in the in silico molecular docking analysis. The leaf chloroform fraction was the only sample that showed an antitumor potential against colon adenocarcinoma cells (HCT116)

Reporte de un caso: eritema discrómico perstans en paciente con diagnóstico de virus de inmunodeficiencia humana / Case report: dyschromic erythema perstans in a patient diagnosed with human immunodeficiency virus

El eritema discrómico perstans o dermatitis cenicienta, es un trastorno pigmentario de la piel poco común, de etiología desconocida. Se describe el caso de un adulto de 35 años con antecedente de VIH, quien consulta por aparición de lesiones irregulares, bien definidas café-grises localizadas en cuello, área mandibular inferior, espalda y brazos, de borde levemente eritematoso. El diagnóstico de eritema discrómico perstans se realizó con base en los hallazgos clínicos e histopatológicos.

Erythema dyschromicum perstans also known as ashy dermatosis is a rare skin pigmentary disorder of unknown etiology. We describe the case of a 35-year-old man HIV positive who presented irregular, well defined brown-gray lesions with slightly erythematous border located in neck, lower jaw, back and arms. Diagnosis of erythema dyschromicum perstans in this patient was made based on clinical and histopathological criteria.

Familial progressive hyper- and hypopigmentation: a report on a Chinese family and evidence for genetic heterogeneity

Abstract Background: Familial progressive hyper- and hypopigmentation (FPHH) is a rare genodermatosis that is characterized by diffuse hyper- and hypopigmented spots on the skin and mucous membranes. It is caused by a pathogenic mutation of the KITLG gene. Objectives: To investigate the clinical features and mutation of the KITLG gene in a Chinese family with FPHH. Methods: Histopathological and immunohistochemical analysis of lesions from the proband was performed. The KITLG gene was screened for the presence of mutations. Results: A Chinese family containing 14 individuals with FPHH was described, and the proband was a 5-year-old girl showing diffuse hyper- and hypopigmented lesions on her extremities and trunk. Histopathological and immunohistochemical staining for S100 and HMB45 of skin biopsy specimens from the hyperpigmented areas showed a striking increase in melanin throughout the epidermis, especially in the basal cell layer, and staining of hypopigmented area specimens displayed lower levels of melanin in the epidermis. Mutation analysis of the KITLG gene was performed, but no mutation was found. Study limitations: The new pathogenic gene was not found. Conclusion: A family with FPHH was described. Analysis revealed that its members did not have any mutations of the KITLG gene, which provided evidence for genetic heterogeneity of this genodermatosis.

A novel frameshift mutation of Chediak-Higashi syndrome and treatment in the accelerated phase

Chediak-Higashi syndrome (CHS) is a rare autosomal recessive immunodeficiency disease characterized by frequent infections, hypopigmentation, progressive neurologic deterioration and hemophagocytic lymphohistiocytosis (HLH), known as the accelerated phase. There is little experience in the accelerated phase of CHS treatment worldwide. Here, we present a case of a 9-month-old boy with continuous high fever, hypopigmentation of the skin, enlarged lymph nodes, hepatosplenomegaly and lung infection. He was diagnosed with CHS by gene sequencing, and had entered the accelerated phase. After 8 weeks of therapy, the boy had remission and was prepared for allogenic stem cell transplantation.

Do you know this syndrome? Dyspigmentation along the Blaschko lines caused by trisomy 7 mosaicism

Abstract Dyspigmentation along the Blaschko lines is strongly suggestive of a mosaic skin disorder. We report a 9-year-old male patient who presented with swirls and streaks of both hypo and hyperpigmentation involving the entire body. Additionally, he had hypertrichosis, musculoskeletal and minor neurodevelopment abnormalities but no intellectual disability. Cultured fibroblast displayed trisomy 7 mosaicism, which can explain this pigmentary phenotype. Widespread dyspigmentation associated with involvement of other organs should prompt systemic examination to detect additional anomalies and genetic evaluation should be considered, even with normal fetal karyotype.

Bier's spots with onset in childhood

Abstract Bier spots are small, irregular, hypopigmented macules that are usually found on the arms and legs. The macules disappear when the limb is raised. Bier spots have been reported in association with a number of conditions but there is no consistent association to specific desease. Although they usually affect young adults, we report a case of Bier spots that began in childhood. As an asymptomatic and possibly transitional condition, the disease does not require treatment.

Chemical leukoderma induced by dimethyl sulfate

Abstract Chemical leukoderma occurs due to the toxic effect of a variety of chemical agents. Mechanisms include either destruction or inhibition of melanocytes. We report two male patients (36 and 51 years old) who presented with multiple hypopigmented macules and patches on the neck, wrist, and legs after exposure to dimethyl sulfate in a chemical industry. Physical examination revealed irregular depigmentation macules with sharp edges and clear hyperpigmentation around the lesions. History of repeated exposure to a chemical agent can help the clinical diagnosis of chemical leukoderma. This diagnosis is very important for prognosis and therapeutic management of the disease.

Hypopigmented macules of the limbs in two sisters: report on familial Bier spots

AbstractBier spots are asymptomatic, small, irregular, hypopigmented macules characterized by a normal histological appearance, which are usually found on the arms and legs of young adults. We describe the simultaneous presence of Bier spots in two siblings. This finding is unusual since, to the best of our knowledge, concurrent familial cases have never been reported in the literature.

Dermatosis por neblina ácida: a propósito de un caso / Dermatosis be caused by exposure to acid mist: a case report

Las dermatosis laborales son patologías frecuentes en la práctica clínica y producen un problema importante en la salud de los pacientes, siendo la dermatitis de contacto ocupacional la más frecuente. A continuación presentamos el caso clínico de un trabajador de la minería expuesto en su ambiente laboral a un aerosol ácido, llamado neblina ácida, presentando una hipopigmentación post inflamatoria secundaria a la exposición a éste. Tanto el proceso diagnóstico, como la prevención de las dermatitis de contacto laboral, debe ser un proceso riguroso, ya que su pronóstico es variable, incluso cuando se logre evitar la exposición al agente causal.

Work-related dermatoses are frequent pathologies in the clinical practice and produce a major health- problem, being the occupational contact dermatitis the most frequent disease. We study the case of a mining worker exposed in his work enviroment to an acid aerosol, called acid mist, presenting a post inflammatory hypopigmentation after the exposure to this acid. The diagnosis process, as well as the prevention of occupational contact dermatitis must be rigorous, since their prognosis is variable, despite of avoiding the exposure to the causative agent.

Hypopigmented mycosis fungoides: a review of its clinical features and pathophysiology / Micose fungoide hipocromiante: uma revisao de seus aspectos clinicos e fisiopatologicos

Several distinct clinical forms of mycosis fungoides have been described. Hypopigmented mycosis fungoides should be regarded as a subtype of mycosis fungoides, insofar as it presents some peculiar characteristics that contrast with the clinical features of the classical form. Most patients with hypopigmented mycosis fungoides are younger than patients typically diagnosed with classical mycosis fungoides. In addition to typical dark-skinned individuals impairment, hypopigmented mycosis fungoides has also been described in Asian patients. The prognosis for hypopigmented mycosis fungoides is much better than for classical mycosis fungoides: hypopigmented mycosis fungoides is diagnosed when there are only patches of affected skin, and lesions usually will not progress beyond terminal stages, although they can persist for many years. Diagnosis should involve clinicopathologic correlation: skin biopsy analysis often reveals intense epidermotropism, characterized by haloed, large, and atypical CD8+ lymphocytes with convoluted nuclei, in contrast to mild to moderate dermal lymphocytic infiltrate. These CD8+ cells, which participate in T helper 1-mediated immune responses, prevent evolution to mycosis fungoides plaques and tumors and could be considered the main cause of the inhibition of melanogenesis. Therefore, hypopigmentation could be considered a marker of good prognosis for mycosis fungoides.

Ultimamente diferentes formas clínicas da micose fungoide têm sido descritas. A micose fungoide hipocromiante pode ser considerada um subtipo da micose fungoide, apresentando algumas características peculiares que contrastam com os achados da forma clássica da micose fungoide. A maioria dos pacientes com micose fungoide hipocromiante são mais jovens que aqueles acometidos pela micose fungoide clássica. Esta variante é descrita principalmente em indivíduos melanodérmicos (afroamericanos e asiáticos). O prognóstico é melhor que o observado para a forma clássica: ao diagnóstico, os pacientes apresentam somente "patches", que tendem a perdurar por longos períodos, sem evolução para estágios mais avançados. O diagnóstico é feito através da correlação clinicopatológica: biópsia da lesão cutânea frequentemente revela intenso epidermotropismo, caracterizado por linfócitos CD8+ atípicos, grandes, com halo e núcleo convoluto, contrastando com o infiltrado dérmico leve a moderado. Estas células CD8+, que participam do perfil de resposta T helper-1, impediriam a evolução da doença para o desenvolvimento de placas infiltradas e tumores, além de determinar a inibição da melanogênese nas lesões hipocrômicas. Portanto, a hipocromia poderia ser considerada um marcador de bom prognóstico na micose fungoide.

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.

Patrones clínicos de mosaicismos pigmentarios. Nuestra experiencia / Clinical patterns of pigmentary mosaicism: our experience

Los mosaicismos pigmentarios son enfermedades névicas caracterizadas por la presencia en piel de máculas híper o hipopigmentadas de distribución según patrones clínicos preestablecidos. En ocasiones presentan asociaciones extracutáneas que alteran el desarrollo normal del individuo. Presentamos una recopilación de todos los casos de mosaicismos pigmentarios evaluados en nuestro Servicio en laúltima década

Leucodermia sifilítica: expressão rara do secundarismo diagnosticada por exame histopatológico / Leucoderma syphiliticum: a rare expression of the secondary stage diagnosed by histopathology

Biópsias são ocasionalmente necessárias para confirmação diagnóstica de sífilis secundária, normalmente obtida por correlação clínico-sorológica. Entretanto, o exame histopatológico pode oferecer pistas que conduzam a um diagnóstico em casos antes insuspeitos ou de apresentação clínica incomum. Apresentamos um paciente de 35 anos, há dois com lesões acrômicas vitiligoides, para o qual sífilis foi sugerida somente após o exame histopatológico. Alguns aspectos microscópicos observados são discutidos e comparados com dados disponíveis na literatura.

Biopsies are occasionally necessary to confirm the diagnosis of secondary-stage syphilis, currently achieved by clinico-serological correlation. However, histopathologic examination may offer clues that can lead to the diagnosis of the disease in previously unsuspected or unusual cases. We report the case of a 35-year-old male patient with vitiligo-like lesions for two years, whose diagnosis of syphilis was suggested only after histopathologic examination. Some microscopic aspects observed are discussed and compared to data from the literature.

Micosis fungoides hipopigmentada: reporte de siete casos / Hypopigmented mycosis fungoides: report of seven cases

Introducción: La micosis fungoides (MF) es el linfoma cutáneo de células T más frecuente. Se presenta de diversas formas clínicas, siendo la micosis fungoides hipopigmentada una variante clínica poco común, sobre todo en población caucásica. Objetivo general: Describir los hallazgos clínicos y anatomopatológicos de la serie de pacientes con diagnóstico de MF hipopigmentada. Material y método: Estudio descriptivo, retrospectivo. Se analizaron todos los casos de MF hipopigmentada diagnosticados en el período 2004-2009 en tres centros de atención dermatológica. Resultados: Se describen siete pacientes con diagnóstico de MF hipopigmentada; el estudio histológico confirmó el diagnóstico en todos los casos, con un período de seguimiento de seis a 66 meses. El promedio de edad al momento del diagnóstico fue de 20,5 años. La inmunohistoquímica en todos los pacientes fue CD8 +, y seis de los siete pacientes evaluados fueron CD4 +. Cinco de los pacientes fueron manejados con PUVA, y de ellos todos lograron la remisión completa.

Introduction:: Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma. It manifests in diverse clinical forms, and a rare variant of MF is hypopigmented mycosis fungoides. Objective: To describe clinical, pathological and immunohistochemicol findings in a group of 7 hypopigmented MF patients. Material and methods: This is a descriptive and retrospective study. We analyzed all patients with diagnosis of hypopigmented MF during the 2004-2009 period in three dermatological centers. Results: We describe 7 patients diagnosed with hypopigmented MF Histological biopsies confirmed the diagnosis in all cases. The follow-up period was 6 to 66 months. The mean age at diagnosis was 20.5 years. Immunohistochemically, all patients were CD8 + and six were CD4 +. Treatment in five cases was with PUVA, which led to complete clinical remission.

Máculas hipopigmentadas vitiligóides e tumor do infundíbulo folicular / Vitiligoid hypopigmented macules and tumor of the follicular infundibulum

Tumor do infundíbulo folicular é lesão cutânea incomum, com várias formas de apresentação clínica. Destacam-se as formas solitária e eruptiva/múltipla. A primeira apresenta-se como lesão papulonodular descamativa em região de cabeça e pescoço. A segunda, alvo deste relato, é mais rara, ocorrendo em face, pescoço e porção superior do tronco como lesões maculares pardas, avermelhadas ou, mais frequentemente, hipopigmentadas, podendo levar ao diagnóstico diferencial com lesões vitiligóides. A apresentação microscópica das diferentes formas clínicas é similar. Neste artigo, relatamos um caso de tumor do infundíbulo folicular múltiplo/eruptivo apresentando-se como máculas vitiligóides faciais em homem de 35 anos.

Tumor of the follicular infundibulum is an uncommon cutaneous lesion with different forms of clinical presentation, namely solitary and multiple/eruptive variants. The former shows predilection for head and neck and presents as a papulonodular scaly tumor. The latter is less frequent and occurs on facial, neck and upper chest areas as brown, reddish or more commonly hypopigmented macules leading to the differential diagnosis with vitiligo. The different clinical forms share the same histopathologic aspect. The present report describes a case of the multiple variant of tumor of the follicular infundibulum disclosing facial vitiligoid macules in a 35-year-old male patient.

Adult onset, hypopigmented solitary mastocytoma: report of two cases.

Solitary mastocytoma is known to occur predominantly in children below 2 years of age and onset in adulthood is rare. Lesions are hyperpigmented in the majority of cases owing to the stimulation of melanin synthesis by mast cell growth factor. We hereby report two patients with adult onset solitary mastocytoma presenting as hypopigmented plaque. The first case was a 24-year-old man who presented with a plaque on the back of the neck of 5 years duration. The second case was a 30-year-old man who had a well-defined solitary, oval 3 x 2.5 cm plaque on the nape of the neck. Stroking of lesion resulted in a wheal with flare (Darier's sign) in both cases. Systemic examination was within normal limits in both cases. Histopathology revealed a dense toluidine blue-positive infiltrate of mast cells in the upper dermis in both cases.