Síndrome de Laugier-Hunziker, reporte de un caso y revisión de la literatura / Laugier-Hunziker syndrome, case report and literature review

El síndrome de Laugier-Hunziker (SLH) es un trastorno de hiperpigmentación macular benigno adquirido que se caracteriza por la presencia de máculas hiperpigmentadas policrómicas, de superficie plana, con morfología lenticular oval, de márgenes definidos; parte importante de éste es el desarrollo de melanoniquia longitudinal. Su principal diagnóstico diferencial es el síndrome de Peutz-Jeghers, ya que este último tiende a desarrollar neoplasias malignas en el tracto digestivo. Es importante que el estomatólogo conozca el SLH, con la finalidad de poder diagnosticarlo y diferenciarlo de otras entidades, particularmente de aquellas de carácter maligno (AU)

Laugier-Hunziker syndrome (LHS) is an acquired benign macular hyperpigmentation disorder, characterized by the presence of polychromic hyperpigmented macules, with a smooth surface, with oval lenticular morphology, with defined margins; An important part of this is the development of longitudinal melanonychia. Its main differential diagnosis is Peutz-Jeghers syndrome, since it tends to develop malignant neoplasms in the digestive tract. It is important that the Stomatologist knows the LHS, in order to be able to diagnose it and differentiate it from other entities, even those of a malignant nature (AU)

Avaliação da rotulagem de cosméticos clareadores de pele comercializados em Juazeiro, Bahia, Brasil / Evaluation of the labeling of skin lightening cosmetics marketed in Juazeiro, Bahia, Brazil

A hiperpigmentação da pele, principalmente na região facial, resulta em um incômodo estético que afeta a qualidade de vida do indivíduo, levando a busca por produtos clareadores. Este estudo avaliou a conformidade dos rótulos de cosméticos comercializados como "produtos clareadores de pele", bem como a existência de substâncias clareadoras proibidas neste tipo de produto. Foi realizada uma análise transversal descritiva qualitativa no período de abril a maio de 2022, em busca por cosméticos comercializados em estabelecimentos farmacêuticos e lojas de produtos cosméticos localizadas no município de Juazeiro/BA. Foram selecionados 18 produtos e os desvios de rotulagem identificados com base na legislação utilizada vigente à época do estudo, foram: ausência de informações sobre advertências/restrições de uso e número de registro incompleto, equivalente a 16,7% (n = 3) das amostras. A hidroquinona, proibida nesse tipo de produto, foi encontrada em um cosmético (5,5%). Embora a maioria das amostras analisadas esteja em conformidade com as exigências legais, os resultados evidenciam descumprimentos, indicando a necessidade de uma fiscalização mais rigorosa a fim de evitar possíveis danos à saúde do usuário. (AU)

Skin hyperpigmentation, particularly in the facial region, can be an aesthetic nuisance that affects an individual's quality of life, leading them to seek out whitening products. This study evaluated the compliance of cosmetics labels marketed as "skin lightening products", and assessed the presence of whitening substances prohibited in this type of product. A qualitative, descriptive, cross-sectional analysis was conducted between April and May 2022 in Juazeiro, Bahia, Brazil, focusing on cosmetics sold in pharmaceutical establishments and cosmetic product stores. Eighteen products were selected, and labeling deviations identified based on the legislation in force at the time of the study. These included a lack of information on warnings/use restrictions and incomplete registration numbers, affecting 16.7% (n = 3) of the samples. Hydroquinone, prohibited in this type of product by the legislation, was detected in one cosmetic (5.5%). Although most of the analyzed samples comply with legal requirements, the observed non-compliance highlights the need for more stringent inspection to prevent potential harm to user's health. (AU)

Avaliação da rotulagem de cosméticos clareadores de pele comercializados em Juazeiro, Bahia, Brasil / Evaluation of the labeling of skin lightening cosmetics marketed in Juazeiro, Bahia, Brazil

A hiperpigmentação da pele, principalmente na região facial, resulta em um incômodo estético que afeta a qualidade de vida do indivíduo, levando a busca por produtos clareadores. Este estudo avaliou a conformidade dos rótulos de cosméticos comercializados como "produtos clareadores de pele", bem como a existência de substâncias clareadoras proibidas neste tipo de produto. Foi realizada uma análise transversal descritiva qualitativa no período de abril a maio de 2022, em busca por cosméticos comercializados em estabelecimentos farmacêuticos e lojas de produtos cosméticos localizadas no município de Juazeiro/BA. Foram selecionados 18 produtos e os desvios de rotulagem identificados com base na legislação utilizada vigente à época do estudo, foram: ausência de informações sobre advertências/restrições de uso e número de registro incompleto, equivalente a 16,7% (n = 3) das amostras. A hidroquinona, proibida nesse tipo de produto, foi encontrada em um cosmético (5,5%). Embora a maioria das amostras analisadas esteja em conformidade com as exigências legais, os resultados evidenciam descumprimentos, indicando a necessidade de uma fiscalização mais rigorosa a fim de evitar possíveis danos à saúde do usuário.

Skin hyperpigmentation, particularly in the facial region, can be an aesthetic nuisance that affects an individual's quality of life, leading them to seek out whitening products. This study evaluated the compliance of cosmetics labels marketed as "skin lightening products", and assessed the presence of whitening substances prohibited in this type of product. A qualitative, descriptive, cross-sectional analysis was conducted between April and May 2022 in Juazeiro, Bahia, Brazil, focusing on cosmetics sold in pharmaceutical establishments and cosmetic product stores. Eighteen products were selected, and labeling deviations identified based on the legislation in force at the time of the study. These included a lack of information on warnings/use restrictions and incomplete registration numbers, affecting 16.7% (n = 3) of the samples. Hydroquinone, prohibited in this type of product by the legislation, was detected in one cosmetic (5.5%). Although most of the analyzed samples comply with legal requirements, the observed non-compliance highlights the need for more stringent inspection to prevent potential harm to user's health.

Hiperpigmentação Mucocutânea Associada à Terapia de Hidroxiureia em Paciente com Trombocitemia Essencial: Relato de Caso / Mucocutaneous Hyperpigmentation Associated with Hydroxyurea Therapy in a Patient with Essential Thrombocythemia: Case Report

Introdução: A hiperpigmentação mucocutânea é uma condição dermatológica que pode estar relacionada a tratamentos quimioterápicos, a exemplo das terapias com uso de hidroxiureia (HU). A HU é um fármaco citostático de amplo uso nas doenças mieloproliferativas e compõe a principal linha de tratamento da trombocitemia essencial (TE). O presente estudo tem por objetivo relatar um caso raro de hiperpigmentação mucocutânea em um paciente com TE. Relato do caso: Paciente do sexo masculino, 68 anos de idade, 89 kg, com diagnóstico de TE, em uso de HU 2 g/dia. Com três meses de terapia, apresentou lesões hiperpigmentadas de coloração acastanhadas em pele das mãos e mucosa oral (língua). Em decisão partilhada com o médico-assistente, o paciente optou pela continuação do uso do medicamento. Após seis anos de acompanhamento, as lesões mantêm-se estáveis. Conclusão: A hiperpigmentação mucocutânea associada à terapia com HU é um evento benigno secundário ao uso do fármaco e não exige a interrupção de uso, porém, sua retirada, ou redução das doses, geralmente leva à diminuição ou ao desaparecimento das lesões.

Introduction: Mucocutaneous hyperpigmentation is a dermatological condition that may be related to chemotherapy treatments, such as therapies using hydroxyurea (HU). HU is a cytostatic drug widely used in myeloproliferative diseases and is the main line of treatment for essential thrombocythemia (ET). The present study aims to report a rare case of mucocutaneous hyperpigmentation in a patient with ET. Case report: Male patient, 68 years old, 89 kg, diagnosed with ET using HU 2 g/day. After three months of therapy, he presented hyperpigmented brownish-colored lesions on the hands and oral cavity (tongue). In a decision shared with the assistant physician, the patient chose to continue using the drug. After six years of follow-up, the lesions remain stable. Conclusion: Mucocutaneous hyperpigmentation associated with HU therapy is a benign event secondary to the use of the drug and does not require discontinuation of use, however, its withdrawal or dose reduction usually leads to the reduction or disappearance of the lesions

Introducción: La hiperpigmentación mucocutánea es una condición dermatológica que puede estar relacionada con tratamientos de quimioterapia, como las terapias con hidroxiurea (HU). La HU es un fármaco citostático ampliamente utilizado en enfermedades mieloproliferativas y es la principal línea de tratamiento de la trombocitemia esencial (TE). El presente estudio tiene como objetivo reportar un caso raro de hiperpigmentación mucocutánea en un paciente con TE. Informe del caso: Paciente masculino de 68 años, 89 kg, diagnosticado de TE mediante HU 2 g/día. A los tres meses de tratamiento presenta lesiones hiperpigmentadas de color pardusco en manos y cavidad oral (lengua). En una decisión compartida con el médico asistente, el paciente optó por continuar usando el medicamento. Tras seis años de seguimiento, las lesiones se mantienen estables. Conclusión: La hiperpigmentación mucocutánea asociada a la terapia con HU es un evento benigno secundario al uso del fármaco y no requiere la suspensión de su uso, sin embargo, su retirada o reducción de dosis suele conducir a la reducción o desaparición de las lesiones.

Comparative Evaluation of Clinical Efficacy of Diode LASER and Cryosurgery for Gingival Pigmentation: A Split-Mouth Randomized Clinical Study

ABSTRACT Objective: To compare and evaluate the clinical efficacy of diode laser and cryosurgery for treating melanin pigmentation of gingiva. Material and Methods: A total of twenty-five subjects with physiological gingival pigmentation on the facial aspect of both maxillary and mandibular anterior arches (50 sites), both male and female, with an average age ranging from 18-35 years, participated in the study. The sites were randomly divided into Group I: depigmentation by Laser and Group II: depigmentation by Cryosurgery. The following parameters were assessed for the evaluation of treatment results: Melanin Oral Pigmentation Index (PI), Visual Analogue Scale (VAS) for pain evaluation and Healing index (HI). The data collected was statistically evaluated. Results: On intergroup comparison, there was no statistical difference in the score from baseline (p>0.05); however, a statistically significant difference was seen at the end of 1 year (p<0.05). Moreover, 57-60% of arches showed recurrence of pigmentation in the laser group whereas; only 12.7-17% recurrence was seen in the cryosurgery group at the end of the first year. Conclusion: Treatment of gingival hyperpigmentation with laser and cryosurgery shows a marked improvement of gingival pigmentation in both groups, but the cryosurgery depigmentation sites showed more sustainability.

Papilomatosis confluente y reticulada de Gougerot-Carteaud / Confluent and reticulated papillomatosis of Gougerot-Carteaud

La papilomatosis confluente y reticulada es una dermatosis infrecuente, benigna, de curso crónico y etiopatogenia desconocida. Actualmente, se acepta que se debe a un trastorno de la queratinización. Se caracteriza por máculas y pápulas hiperpigmentadas, que coalescen en el centro y adoptan un patrón reticular en la periferia. Se la puede confundir con otras patologías, como la pitiriasis versicolor y la acantosis nigricans. Con la administración de minociclina por vía oral suelen obtenerse excelentes resultados. Se comunica el caso de un paciente con diagnóstico de papilomatosis confluente y reticulada. Se describen sus características clínicas, criterios diagnósticos y trata-miento instaurado.

Confluent and reticulated papillomatosis is an infrequent, benign dermatosis of chronic course and unknown etiopathogenesis. Currently it is accepted that this dermatosis is due to a keratinization disorder. It is characterized by hyperpigmented macules and papules that coalesce in the center and adopt a reticular pattern in the periphery. It can be confused with other pathologies such as pityriasis versicolor and acanthosis nigricans. Oral minocycline usually shows excellent results. The present work reports a case of a patient with a diagnosis of confluent and retic-ulated papillomatosis, describing its clinical characteristics, diagnostic criteria and established treatment.

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)

Melanosis gingival: diagnóstico y terapéutica de su implicación estética. Revisión de la literatura / Gingival Melanosis: Diagnosis and Therapy of Its Aesthetic Involvement. Literature Review

ABSTRACT: Gingival hyperpigmentation is produced by excessive melanin deposit, generating a dark gum coloring. Although it does not constitute a health issue, in some cases it usually represents an aesthetic problem that can affect psychologically, for which there are currently several treatment alternatives such as: surgery with scalpel, laser therapy, abrasion, cryosurgery, electrosurgery, among others. The aim of this literature review was to analyze the available information about gingival melanosis and the therapeutics that can be applied to improve the appearance of patients with this condition. Articles in English and Spanish, published during the period 2000-2020 in the PubMed, Medline, Scielo, Cochrane and Lilacs databases, were reviewed. It was concluded that the selection of the technique will depend on each particular case, however, the laser is the most relevant.

RESUMEN: La hiperpigmentación gingival se produce por el depósito excesivo de melanina, generando una coloración oscura de la encía. Aunque no constituye un inconveniente para la salud, en algunos casos suele representar un problema estético que puede afectar psicológicamente, por lo cual, en la actualidad existen diversas alternativas de tratamiento como: cirugía con bisturí, terapia láser, abrasión, criocirugía, electrocirugía, entre otros. El objetivo de esta revisión de la literatura fue analizar la información disponible acerca de la melanosis gingival y la terapéutica que puede ser aplicada para mejorar el aspecto de los pacientes con esta condición. Se revisaron artículos en inglés y español, publicados durante el período 2000-2020 en las bases de datos PubMed, Medline, Scielo, Cochrane y Lilacs. Se concluyó que la elección de la técnica dependerá de cada caso en particular, sin embargo, el láser es el más destacado.

Síndrome de Peutz-Jeghers, telangiectasias y prolapso de la válvula mitral. Presentación de caso y asesoramiento genético / Peutz-Jeghers syndrome, telangiectasias, and mitral valve prolapse. Case presentation and genetic counseling

Introducción: El síndrome de Peutz-Jeghers se caracteriza por hiperpigmentación mucocutánea y hamartomas gastrointestinales que pueden aparecer desde el estómago hasta el ano. Tiene un patrón de herencia autosómico dominante y expresividad variable. El diagnóstico se basa en los hallazgos clínicos y la apariencia histológica de los pólipos. No ha sido reportado hasta ahora asociación de esta entidad a telangiectasias y prolapso de la válvula mitral. Objetivo: Describir los hallazgos que permitieron establecer el diagnóstico de Síndrome de Peutz-Jeghers en un paciente y brindar asesoramiento genético. Presentación del caso: Paciente masculino de 36 años de edad con antecedentes de prolapso de la válvula mitral que acude a consulta de genética clínica con su esposa para solicitar asesoramiento genético, debido a que tienen una hija con diagnóstico de Síndrome de Peutz-Jeghers y desean conocer el riesgo de tener otro hijo afectado. Al examen físico se observa mácula hiperpigmentada en labio inferior y varias de estas en encías. Con tales hallazgos y el antecedente de tener la hija Síndrome de Peutz-Jeghers se emite el mismo diagnóstico en el padre. Como dato de interés se constatan en este individuo múltiples telangiectasias en tórax, cuello y espalda. Los estudios realizados en busca de la causa de estas fueron negativos. Conclusiones: Los antecedentes y los hallazgos encontrados en el paciente permitieron realizar el diagnóstico de Peutz-Jeghers y brindar asesoramiento genético. Se presenta el primer reporte de esta enfermedad asociada a telangiectasias y prolapso de la válvula mitral en la literatura científica(AU)

Introduction: Peutz-Jeghers syndrome is characterized by mucocutaneous hyperpigmentation and gastrointestinal hamartomas that can appear from the stomach to the anus. It has an autosomal dominant inheritance pattern and variable expressiveness. The diagnosis is based on clinical findings and histological appearance of the polyps. No association between this entity and telangiectasias and mitral valve prolapse has been reported so far. Objective: To describe the findings that made it possible to establish the diagnosis of Peutz-Jeghers syndrome in a patient and to provide genetic counseling. Case presentation: Thirty-six-year-old male patient with a history of mitral valve prolapse who attends a clinical genetics consultation with his wife to request genetic counseling due to the fact that their daughter was diagnosed with Peutz-Jeghers Syndrome and they want to know about the risk of having another affected child. On physical examination, a hyperpigmented macule on the lower lip and several of these on the gums were observed. With such findings and the antecedent of having a daughter with Peutz-Jeghers syndrome, the same diagnosis is made in the father. As data of interest, multiple telangiectasias on the thorax, neck and back were found in this individual. The studies carried out to identify the same cause were negative. Conclusions: The history and findings in this patient allowed us to make the diagnosis of Peutz-Jeghers syndrome as well as to provide genetic counselling. The first report of this disease associated with telangiectasias and mitral valve prolapse is presented in the scientific literature(AU)

Escleroterapia con polidocanol en insuficiencia venosa superficial CEAP C1 / Sclerotherapy with polidocanol in superficial venous insufficiency CEAP C1

Insuficiencia venosa es definida como la patología que causa síntomas en los miembros inferiores, incluyendo edema, hiperpigmentación, lipodermatoesclerosis y ulceración e implica una anormalidad funcional del sistema venoso. Objetivo: Se compararon los resultados en cuanto a complicaciones y satisfacción reportada por las pacientes, con la Escleroterapia con Polidocanol utilizando aire y agua como diluyente. Métodos: Se incluyeron en el estudio a 60 pacientes del sexo femenino con diagnóstico de Insuficiencia Venosa Superficial CEAP C1, divididas en dos grupos, a las cuales se les aplicó Escleroterapia con Polidocanol mezclado con agua y aire. Se realizó seguimiento durante cuatro semanas donde se recogió la información del paciente con respecto a la mejoría de los síntomas antes de iniciar el tratamiento y los efectos adversos del Polidocanol con ambas terapias. Resultados: Dentro de las complicaciones de ambos tratamientos únicamente fue reportado el Matting en rango leve durante la primera semana. El único síntoma reportado como severo fue Dolor en la escleroterapia con polidocanol mezclado con aire. Los demás signos síntomas fueron reportados dentro del rango de moderado el cual descendió hasta leve entre la segunda y tercera semana, no encontrando reportes a la cuarta semana. La única complicación reportada fue el Matting en la escleroterapia con polidocanol mezclado con aire. Conclusión: No se encontraron diferencias significativas en grado de satisfacción con la eficacia del tratamiento, los efectos adversos, la forma en que se administra el medicamento entre ambos tratamientos de escleroterapia, oscilando los rangos de satisfacción entre el 43 al 70%. (AU)

Venous insufficiency is defined as the pathology that causes symptoms in the lower limbs, including edema, hyperpigmentation, lipodermatosclerosis and ulceration and implies a functional abnormality of the venous system. Objective: The results in terms of complications and satisfaction reported by the patients were compared with Sclerotherapy with Polidocanol using air and water as diluent. Methods: 60 female patients with a diagnosis of Superficial Venous Insufficiency CEAP C1 were included in the study, divided into two groups, to which Sclerotherapy with Polidocanol mixed with water and air was applied. A follow-up was carried out for four weeks where information from the patient was collected regarding the improvement of symptoms before starting treatment and the adverse effects of Polidocanol with both therapies. Results: Within the complications of both treatments, only Matting was reported in a mild range during the first week. The only symptom reported as severe was pain in sclerotherapy with polidocanol mixed with air. The other signs and symptoms were reported within the moderate range, which decreased to mild between the second and third week, finding no reports at the fourth week. The only complication reported was Matting in sclerotherapy with polidocanol mixed with air. Conclusion: No significant differences were found in the degree of satisfaction with the efficacy of the treatment, the adverse effects, the way in which the drug is administered between both sclerotherapy treatments, the satisfaction ranges ranging from 43 to 70%. (AU)

Importancia de las características clínicas y de laboratorio en el diagnóstico de las infecciones concomitantes por dengue y chikungunya: reporte de un caso probable / Importance of clinical and laboratory characteristics in the diagnosis of dengue and chikungunya co-infection: Probable case report

Se presenta el caso de una mujer de 38 años que consultó inicialmente por fiebre indiferenciada. A pesar de que el cuadro clínico evolucionó con manifestaciones clínicas de dengue con signos de alarma y de que la detección de IgM antidengue en una sola muestra indicaba que se trataba de un caso probable que había podido ocurrir durante los tres meses anteriores, la paciente consultó de forma reiterada, pues no presentaba una mejoría significativa. En el décimo día del inicio de los síntomas, se observó edema simétrico en múltiples articulaciones acompañado de dolor, así como lesiones hiperpigmentadas en el surco nasogeniano. Se confirmó el diagnóstico de chikungunya por la presencia de anticuerpos IgM. Aunque puede pasar desapercibida, en los países endémicos para dengue y chikungunya existe la posibilidad de la infección concomitante, la cual puede agravar la evolución clínica de cada una de estas enfermedades. Por ello, es necesario que el médico considere las características clínicas y de laboratorio de ambas enfermedades para diagnosticar su presencia simultánea, garantizar un manejo adecuado y minimizar las complicaciones.

We report the case of a 38-year-old woman who initially consulted for an undifferentiated fever. Although her clinical condition evolved with signs and symptoms compatible with dengue with alarm signs and that the anti-dengue IgM detection in a single sample indicated it was a probable case that could have happened during the previous three months, the patient kept consulting due to little improvement. On the tenth day after the onset of symptoms, she presented with painful polyarticular symmetric edema, as well as hyperpigmented lesions in the nasolabial fold. Chikungunya diagnosis was confirmed by the presence of IgM antibodies. In endemic countries for dengue and chikungunya, the possibility of co-infection exists, but it may go unnoticed. On the other hand, the co-infection may worsen the clinical course of these diseases. Therefore, physicians should evaluate the clinical and laboratory characteristics of both infections to be able to diagnose the coinfection for adequate management and to minimize complications.

Incontinentia pigmenti: genodermatosis multisistémica / Incontinentia pigmenti: multisistemic genodermatosis

Resumen La incontinentia pigmenti es una genodermatosis ligada al cromosoma X, generalmente letal en los hombres. Está causada por una mutación con pérdida de función en el gen IKBKG (inhibitor of kappa polypeptide gene enhancer in B cells kinase gamma), que impide que la proteína NFкβ (nuclear factor kappa-light-chain-enhancer of activated B cells) migre al núcleo y comience la transcripción de factores que amplifican la respuesta inmunitaria y previenen la apoptosis. Por tanto, las células mutantes se vuelven vulnerables a la apoptosis cuando son expuestas a citocinas y provocan vaso-oclusión e isquemia de tejidos como la piel, el sistema nervioso central y la retina. Las lesiones dermatológicas son características; se distribuyen a lo largo de las líneas de Blaschko, las cuales siguen el patrón de migración de las células de la piel en la embriogénesis, y ocurren en el 100% de los pacientes. Las manifestaciones cutáneas aparecen en una secuencia de cuatro fases que inicia desde el nacimiento: vesicular, verrucosa, hiperpigmentada e hipopigmentada. Estas lesiones son relevantes, puesto que orientan al clínico hacia el diagnóstico. Además, se acompañan de anomalías neurológicas, como crisis convulsivas, y múltiples manifestaciones oftalmológicas, como el desprendimiento de la retina. Los pacientes con incontinentia pigmenti, pero sin compromiso oftalmológico o neurológico clínicamente significativo, tienen un pronóstico bueno y una esperanza de vida normal. Las anomalías que se presentan son permanentes, lo que puede generar preocupación en los pacientes.

Abstract Incontinentia pigmenti is an X-linked genodermatosis generally lethal in males; thus, it presents almost exclusively in females. It is caused by a loss-of-function mutation in the IKBKG (inhibitor of kappa polypeptide gene enhancer in B cells, kinase gamma) gene that prevents the NFкβ (nuclear factor kappa-light-chain-enhancer of activated B cells) protein from migrating to the nucleus to begin the transcription of factors that amplify the immune response and prevent apoptosis. Consequently, mutant cells become vulnerable to apoptosis when exposed to cytokines and, in turn, lead to vaso-occlusion and ischemia of tissues, such as the skin, the central nervous system and the retina. Dermatological lesions are characteristic and occur in 100% of patients; they are distributed along Blaschko lines, which follow the pattern of migration of skin cells in embryogenesis. The cutaneous manifestations follow a sequence of four phases since birth: vesicular, verrucous, hyperpigmented and hypopigmented. These lesions are relevant for the disease because they guide the clinician towards the diagnosis. Additionally, they are accompanied by neurological abnormalities, such as seizures, and multiple ophthalmological manifestations, such as retinal detachment. Incontinentia pigmenti patients with no clinically significant ophthalmic or neurological compromise have a good prognosis and a normal life expectancy. The abnormalities present are permanent, which can be a cause of concern for the patients.

Newborn transient patterned hyperpigmentation and anophthalmia / Patrón de hiperpigmentación transitoria del recién nacido y anoftalmia

Abstract Background: Transient pigmentary lines of the newborn are uncommon cutaneous lesions of unknown etiology. To date, only a few cases have been described. Case report: A patient with a combination of transient pigmentary lines and ocular malformation is described. Molecular analysis of the SRY-box 2 (SOX2) and MIFT genes was conducted to rule out any monogenetic etiology. Conclusions: Worldwide, this is the eighth case of transient pigmentary lines of the newborn reported, and the first associated with anophthalmia. No mutations in the analyzed genes (SOX2 and MIFT) were identified. Therefore, somatic mutations could be responsible for this anomaly.

Resumen Introducción: Las líneas transitorias pigmentarias del recién nacido son lesiones cutáneas poco comunes. A la fecha, pocos casos se han descrito. Caso clínico: Paciente neonato con la combinación de líneas transitorias hiperpigmentadas y una malformación ocular. Se realizó secuenciación molecular de los genes SOX2 y MIFT para descartar una etiología monogénica. Conclusiones: En todo el mundo, este es el octavo caso reportado de líneas transitorias hiperpigmentadas del recién nacido, y el primero asociado con anoftalmia. No se identificaron mutaciones en los genes estudiados (SOX2 y MIFT). Por lo tanto, las mutaciones somáticas pueden ser la causa de la afección.

Dermatosis Terra Firma-Forme / Terra Firma-Forme Dermatosis

RESUMEN Presentamos un caso típico de Dermatosis Terra Firma-Forme en un adolescente sano de 13 años de edad, visto recientemente en el Servicio de Dermatología de nuestro hospital. Con este caso queremos mostrar las características clínicas de esta dermatosis que con frecuencia no es correctamente diagnosticada o bien pasa desapercibida durante años lo que origina preocupación y ansiedad en el paciente además de pruebas diagnósticas innecesarias. Su diagnóstico es clínico apoyado en la dermatoscopía y el tratamiento sencillo, presentando escasas recidivas.

SUMMARY We report a typical case of a Terra Firma-Forme Dermatosis in a 13-year-old healthy male recently seen in the Dermatology Department of our hospital. The aim of the authors is to show clinical features of this frequently misdiagnosed and underreported dermatosis causing concern and anxiety in the patient as well as unnecessary diagnostic tests. Its diagnosis is clinical supported by dermoscopy and its simple treatment presents few recurrences.

Hiperpigmentación cutánea secundaria a déficit de vitamina B12 en un lactante / Cutaneous hyperpigmentation secondary to vitamin B12 deficiency in an infant

La hiperpigmentación cutánea es una manifestación clínica poco frecuente del déficit de vitamina B12 (cobalamina). Su causa no se conoce con exactitud, y existen varias teorías en su etiopatogenia.Se presenta a una niña de 8 meses de edad, con antecedentes de retraso neuromadurativo, hipotonía, anemia y neutrope-nia, derivada a nuestro Servicio por hiperpigmentación cutá-nea del dorso de las manos y los pies de 3 meses de evolución. Ante la sospecha clínica de déficit de vitamina B12, se realizó un análisis de laboratorio, en el que se constató una marcada disminución de los niveles séricos de cobalamina, por lo que se indicó tratamiento sustitutivo con esta.Se destaca la importancia de la presunción diagnóstica de déficit de vitamina B12 como causa de hiperpigmentación cutánea y su resolución luego de la instauración del tratamiento adecuado.

Cutaneous hyperpigmentation is a rare clinical feature of vi-tamin B12 (cobalamin) deficiency. The cause is unknown and there are different hypothesis about the pathogenesis of the hyperpigmentation.We report the case of an 8-month-old girl, with history of neu-romadurative delay, hypotonia, anemia and neutropenia, who was referred to our Service by brownishhyperpigmentation on her hands and feet of 3 months of evolution. Based on the clinical suspicion of vitamin B12 deficiency, we performed a laboratory dosage that showed decreased levels of cobalamin, reason for which replacement therapy was indicated.It highlights the importance of the presumption of vitamin B12 deficiency as a cause of cutaneous hyperpigmentation and its rapid resolution after the establishment of the appro-priate treatment.

Is macular lymphocytic arteritis limited to the skin? Long-term follow-up of seven patients

Abstract Background: Macular lymphocytic arteritis most commonly presents as hyperpigmented macules on the lower limbs. The pathogenesis of this disease is still unclear and there is an ongoing debate regarding whether it represents a new form of cutaneous vasculitis or an indolent form of cutaneous polyarteritis nodosa. Objective: To describe clinical, histopathological, and laboratory findings of patients with the diagnosis of macular lymphocytic arteritis. Methods: A retrospective search was conducted by reviewing cases followed at the Vasculitis Clinic of the Dermatology Department, School of Medicine, University of São Paulo, between 2005 and 2017. Seven patients were included. Results: All cases were female, aged 9-46 years, and had hyperpigmented macules mainly on the legs. Three patients reported symptoms. Skin biopsies evidencing a predominantly lymphocytic infiltrate affecting arterioles at the dermal subcutaneous junction were found, as well as a typical luminal fibrin ring. None of the patients developed necrotic ulcers, neurological damage, or systemic manifestations. The follow-up ranged from 18 to 151 months, with a mean duration of 79 months. Study limitations: This study is subject to a number of limitations: small sample of patients, besides having a retrospective and uncontrolled study design. Conclusions: To the best of the authors' knowledge, this series presents the longest duration of follow-up reported to date. During this period, none of the patients showed resolution of the lesions despite treatment, nor did any progress to systemic vasculitis. Similarities between clinical and skin biopsy findings support the hypothesis that macular lymphocytic arteritis is a benign, incomplete, and less aggressive form of cutaneous polyarteritis nodosa.

Profile of depigmenting cosmetics and dermatological products on the market

The skin is the largest and most exposed organ of the human body, therefore subject to diseases and alteration of its appearance. Among these alterations, the cutaneous hyperchromia may be cited. Currently, the market offers numerous products with depigmenting action to the treatment of such disorders. The aim of this work was to analyze depigmenting products commercialized in establishments in the city of Bento Gonçalves (RS, Brazil) and websites of cosmetic companies. It was found 45 products with depigmenting action and, from these, 59 different active agents were identified. The main active compounds found were kojic acid, arbutin, ascorbic acid, hydroquinone and glycolic acid. Another observed data was that in 78% of the studied products the active substances were being used in combination. The most used vehicles were also studied as a reference to the use of sunscreen in the treatment of cutaneous hyperchromia. The present work had identified in the market a variety of products with depigmentation action and, because of this, it aims to serve as a reference to the healthcare professionals, especially at the prescribing moment, looking for the best results, with regards to treatment efficiency and safety.(AU)

The anti-clockwise spiralization of the linea nigra sign / O sinal do espiralamento anti-horário da linea nigra

ABSTRACT Objective: To describe the morphology of the supra- and infraumbilical linea nigra in puerperal women. Methods: The study was conducted from September 2017 to April 2018, and included 157 puerperal women admitted for childbirth care at the Obstetrics Department of a public maternity hospital of the city of São Paulo (SP), Brazil. The abdomen of subjects was photographed on the first or second day postpartum, with the patient lying symmetrically in dorsal decubitus at a standardized distance. Contrast was slightly adjusted and the morphological pattern of supra and infraumbilical linea nigra in the proximity of the umbilical scar was characterized. The images were independently analyzed by two researchers and only the matching results from both observers were used. Results: Of the 157 observed cases, 139 (88.5%) images provided concordant results between the two researchers. Excluding 41 cases of absence or poor definition of the linea nigra, 98 images were analyzed. Supra- and infraumbilical linea nigra were analyzed separately and classified according to three directions (left, center and right of the umbilical scar). The combination of the supra- and infraumbilical images resulted in the formation of nine distinct patterns, being the most prevalent, in primiparous (72.2%) and multiparous women (50.0%), and the authors named as "anticlockwise spiralization of the linea nigra". Conclusion: The analysis of supra- and infraumbilical linea nigra in puerperal women showed a predominance of what the authors named "anti-clockwise spiralization of the linea nigra sign".

RESUMO Objetivo: Descrever a morfologia da linea nigra supra e infraumbilical em puérperas. Métodos: O estudo foi realizado no período de setembro de 2017 a abril de 2018 e incluiu 157 puérperas admitidas para o parto no Serviço de Obstetrícia de uma maternidade pública da cidade de São Paulo (SP). O abdome das pacientes foi fotografado no primeiro ou segundo dia pós-parto, com a paciente deitada simetricamente em decúbito dorsal a uma distância padronizada. O contraste foi ligeiramente ajustado, e o padrão morfológico da linea nigra supra e infraumbilical na proximidade da cicatriz umbilical foi caracterizado. As imagens foram analisadas independentemente por dois pesquisadores, e apenas os resultados concordantes dos dois observadores foram utilizados. Resultados: Dos 157 casos observados, 139 (88,5%) imagens apresentaram resultados concordantes entre os dois pesquisadores. Excluindo 41 casos de ausência ou má definição da linea nigra, 98 imagens foram analisadas. As linea nigra supra e infraumbilicais foram analisadas separadamente e classificadas de acordo com três direções (esquerda, centro e direita da cicatriz umbilical). A combinação das imagens supra e infraumbilicais resultou na formação de nove padrões distintos, sendo os mais prevalentes nas primíparas (72,2%) e multíparas (50,0%), o que os autores denominaram "espiralamento anti-horário da linea nigra". Conclusão: A análise das linea nigra supra e infraumbilical em puérperas mostrou predominância do que os autores denominaram "sinal do espiralamento anti-horário da linea nigra".