Poroqueratosis de Mibelli en la edad pediátrica / Porokeratosis of Mibelli in pediatric age

Las poroqueratosis son un grupo heterogéneo de trastornos de la queratinización epidérmica, de presentación infrecuente. Se caracterizan clínicamente por pápulas hiperqueratósicas que confluyen y forman placas anulares con un centro atrófico y bordes sobreelevados. Hay seis variantes clínicas. El examen histopatológico evidencia la característica laminilla cornoide. Se presenta el caso de una niña de 2 años con diagnóstico de poroqueratosis de Mibelli y respuesta parcial al tratamiento tópico con tretinoína al 0,025%, que actualmente continúa en seguimiento clínico (AU)

Porokeratosis is a heterogeneous group of disorders in epidermal keratinization. It is an infrequent entity characterized clinically by hyperkeratotic papules that converge forming annular plaques with an atrophic center and raised borders. There are six clinical variants. The histopathological examination evidences the typical cornoid lamella. We present a 2-year-old girl diagnosed with porokeratosis of Mibelli and partial response to topical treatment of tretinoin 0,025%, still under clinical control (AU)

Erythrodermic psoriasis and human immunodeficiency virus: association and therapeutic challenges

Abstract: Erythrodermic psoriasis is a rare but severe type of psoriasis that may be triggered by human immunodeficiency virus infection. We describe the case of a 65-year-old male patient with chronic psoriasis who presents an exacerbation of his condition over a period of two weeks. Because of the severity of his case and subsequent need for systemic therapy, human immunodeficiency virus enzyme immunoassay was performed and tested positive. He thus began antiretroviral therapy combined with acitretin, showing good clinical response after 8 weeks of treatment. There is little evidence regarding the management of erythrodermic psoriasis associated with HIV infection, so antiretroviral therapy and systemic retinoid remain as the first-line treatment.

Efficacy and safety of superficial chemical peeling in treatment of active acne vulgaris

Abstract: Acne vulgaris is an extremely common condition affecting the pilosebaceous unit of the skin and characterized by presence of comedones, papules, pustules, nodules, cysts, which might result in permanent scars. Acne vulgaris commonly involve adolescents and young age groups. Active acne vulgaris is usually associated with several complications like hyper or hypopigmentation, scar formation and skin disfigurement. Previous studies have targeted the efficiency and safety of local and systemic agents in the treatment of active acne vulgaris. Superficial chemical peeling is a skin-wounding procedure which might cause some potentially undesirable adverse events. This study was conducted to review the efficacy and safety of superficial chemical peeling in the treatment of active acne vulgaris. It is a structured review of an earlier seven articles meeting the inclusion and exclusion criteria. The clinical assessments were based on pretreatment and post-treatment comparisons and the role of superficial chemical peeling in reduction of papules, pustules and comedones in active acne vulgaris. This study showed that almost all patients tolerated well the chemical peeling procedures despite a mild discomfort, burning, irritation and erythema have been reported; also the incidence of major adverse events was very low and easily manageable. In conclusion, chemical peeling with glycolic acid is a well-tolerated and safe treatment modality in active acne vulgaris while salicylic acid peels is a more convenient for treatment of darker skin patients and it showed significant and earlier improvement than glycolic acid

Queratolisis punctata / Pitted keratolysis

Se presenta caso de paciente de 13 años, de sexo femenino, con historia de 2 meses de evolución de lesiones aspecto crateriforme en planta de pie izquierdo, acompañado de bromhidrosis, dolor y ardor e impotencia funcional. El cultivo reveló la presencia de Staphylococcus aureus meticilino sensible. Las lesiones mejoraron con tratamiento antibiótico tópico con mupirocina y queratolíticos, quedando como diagnóstico una queratólisis punctata.

A 13-year-old female patient case is presented with a 2 months history of lesions on the left foot, accompanied by bromhydrosis, pain, burning, and functional impotence. Tissue culture revealed the presence of sensitive methicillin Staphylococcus aureus. The lesions improved with topical antibiotic treatment with mupirocin and keratolytics, final diagnosis was punctate keratolysis.

Enfermedad de Darier. Presentación en dos hermanos / Darier's disease. Report in two brothers

La enfermedad de Darier (ED) o disqueratosis folicular fue descripta por Darier y White en 1889. Se trata de una alteración de herencia autosómica dominante, causada por la mutación del gen ATP2A2. Esta mutación interfiere en los procesos de diferenciación y crecimiento celular calcio dependientes. El diagnóstico es clínico e histopatológico. Se manifiesta con pápulas y costras eritematoparduzcas queratósicas, pruriginosas, localizadas principalmente en áreas seborreicas, pápulas blanquecinas en mucosa oral y alteraciones ungueales. Presentamos dos casos de ED en dos hermanos y realizamos una revisión de esta entidad (AU)

Darier's disease (DD) or keratosis follicularis was described by Darier and White in 1889. It is an autosomic dominant inheritance disorder caused by mutation of ATP2A2 gene. This mutation interferes in the processes of cell differentiation and calcium dependent growth. Diagnosis is clinical and histopathological. Clinical features include itchy keratotic papules and crusts, located mainly on seborrheic areas, whitish papules on oral mucosa and nail changes. Two cases of DD in two brothers is reported and a review of this entity is made (AU)

Generalized pustular psoriasis induced by systemic steroid dose reduction

Abstract: Generalized pustular psoriasis, or psoriasis of von Zumbusch, is an acute and severe clinical form of psoriasis, which usually occurs in patients with psoriasis undergoing aggravating factors. In this work, we report the case of a female patient, 70 years old, who developed generalized pustular psoriasis symptoms while reducing the dose of oral corticosteroids, improperly introduced for the treatment of alleged acute generalized exanthematous pustulosis. The differential diagnosis of generalized pustular psoriasis should be made with other pustular dermatoses, such as subcorneal pustulosis, IgA pemphigus and especially with acute generalized exanthematous pustulosis. Personal history of psoriasis and histopathological findings with psoriasiform changes and subcorneal pustule favored the diagnosis. She was treated with acitretin 30 mg / day, progressing to complete regression of the lesions.

Acrokeratosis verruciformis of Hopf - Case report

Abstract: A 54 year-old woman with a 3-year history of rheumatoid arthritis (RA) consulted us because of weight loss, fever and skin eruption. On physical examination, erythematous plaques with a pseudo-vesicular appearance were seen on the back of both shoulders. Histological examination was consistent with rheumatoid neutrophilic dermatosis (RND). After three days of prednisone treatment, the skin eruption resolved. RND is a rare cutaneous manifestation of seropositive RA, characterized by asymptomatic, symmetrical erythematous plaques with a pseudo-vesicular appearance. Histology characteristically reveals a dense, neutrophilic infiltrate with leucocitoclasis but without other signs of vasculitis. Lesions may resolve spontaneously or with RA treatment. This case illustrates an uncommon skin manifestation of active rheumatoid arthritis.

Primary localized cutaneous amyloidosis with lichen and poikiloderma-like lesions and an excellent response to systemic acitretin

Abstract: Primary localized cutaneous amyloidosis is a skin-limited amyloidosis that does not involve internal organs. It is clinically subclassified into 3 general categories and some rare variants. However, there is considerable overlap within the classification. Though there are a variety of therapeutic measures, the treatment is often unsatisfactory, particularly when the disease is severe and extensive. We describe a rare case of primary localized cutaneous amyloidosis with lichen and poikiloderma-like lesions that showed an excellent response to systemic acitretin.

Abordagem cirúrgica de faixas constritivas em Síndrome de Vohwinkel / Surgical correction of constrictive bands in Vohwinkel syndrome

A Síndrome de Vohwinkel ou ceratodermia hereditária mutilante é uma ceratodermia palmoplantar rara, que se manifesta na infância e se torna mais evidente nas fases de adolescência e idade adulta. Doença de herança preferencialmente autossômica dominante, acomete mais mulheres e caucasianos. A hiperceratose palmoplantar difusa, com aparência de favo de mel; as faixas constritivas digitais conhecidas como pseudoainhum, e as placas ceratósicas em forma de estrela-do-mar no dorso de mãos e pés, podendo acometer cotovelos e joelhos, são os achados clínicos característicos da Síndrome. O presente trabalho trata de um relato de caso de uma paciente com Síndrome de Vohwinkel e a terapêutica cirúrgica realizada nas faixas constritivas.

Vohwinkel syndrome, also known as hereditary mutilating keratoderma, is a rare palmoplantar keratoderma that manifests in childhood and becomes more evident in adolescence and adulthood. This preferential autosomal dominant disease affects more women and Caucasians. Its clinical features are diffuse palmoplantar keratoderma with the appearance of honeycomblike constricting rings in the fingers and toes known as pseudo-ainhum, and starfish-shaped keratotic plaques on the dorsal aspect of the hands and feet that can affect the elbows and knees. The present report describes a case report of a patient with Vohwinkel syndrome and surgical correction of the constrictive bands.

Triple nanoemulsion potentiates the effects of topical treatments with microencapsulated retinol and modulates biological processes related to skin aging / Tripla nanoemulsao potencializa os efeitos do tratamento topico com retinol microencapsulado e modula processos biologicos relacionados ao envelhecimento da pele

BACKGROUND: The sum of environmental and genetic factors affects the appearance and function of the skin as it ages. The identification of molecular changes that take place during skin aging provides biomarkers and possible targets for therapeutic intervention. Retinoic acid in different formulations has emerged as an alternative to prevent and repair age-related skin damage. OBJECTIVES: To understand the effects of different retinoid formulations on the expression of genes associated with biological processes that undergo changes during skin aging. METHODS: Ex-vivo skin samples were treated topically with different retinoid formulations. The modulation of biological processes associated with skin aging was measured by Reverse Transcription quantitative PCR (RT-qPCR). RESULTS: A formulation containing microencapsulated retinol and a blend of active ingredients prepared as a triple nanoemulsion provided the best results for the modulation of biological, process-related genes that are usually affected during skin aging. CONCLUSION: This association proved to be therapeutically more effective than tretinoin or microencapsulated retinol used singly. .

FUNDAMENTOS: A soma de fatores genéticos e ambientais afeta a aparência e a funcionalidade da pele ao longo do envelhecimento. O conhecimento a respeito das mudanças moleculares durante o envelhecimento fornece biomarcadores e possíveis alvos para intervenções terapêuticas. O ácido retinoico em diferentes formulações surgiu como uma alternativa para prevenir e reparar os danos da pele associados ao envelhecimento. OBJETIVOS: Avaliar comparativamente os efeitos de diferentes formulações contendo retinoides na expressão de genes associados a processos biológicos que são alterados com o envelhecimento da pele. MÉTODOS: Peles ex vivo foram topicamente tratadas com diferentes retinoides, micro e nanoencapsulados. A modulação dos processos biológicos associados ao envelhecimento da pele foi medida por PCR quantitativa, precedida de transcrição reversa (RT-qPCR). RESULTADOS: A formulação contendo uma mistura de princípios ativos incorporados em uma tripla nanoemulsão e retinol microencapsulado apresentou os melhores resultados de modulação de genes relacionados a processos biológicos que são normalmente alterados durante o envelhecimento da pele. CONCLUSÃO: Essa associação demonstrou uma maior eficácia terapêutica quando comparada ao uso isolado de tretinoína ou retinol microencapsulado. .

NAPSI utilization as an evaluation method of nail psoriasis in patients using acitretin / Utilização do NAPSI como instrumento de avaliação na psoríase ungueal em pacientes com uso da acitretina

BACKGROUND: assessment instruments of nail psoriasis have been published in literature as means to standardize the previous subjective assessments of authors. OBJECTIVES: Using Nail Psoriasis Severity Index for evaluation in patients with plaque psoriasis during treatment with acitretin. METHODS: Thirty volunteers with plaque psoriasis were selected for the study. Twenty patients (8 women and 12 men) completed the study. The initial dose of acitretin was 0.3 mg/kg/day for 30 days and was later raised to 0.5 mg/kg/day. Nail Psoriasis Severity Index was collected in the first evaluation, after 2 and 4 months. Nails of both hands were evaluated. RESULTS: nail lesions were present in all patients at first evaluation. The initial Nail Psoriasis Severity Index median was 20 and the final score 20.5 (2.5% of worsening at the end of the study). No statistically significant difference between the three evaluations was found (X2 = 0.8084, GL = 2, p = 0.6657). Seven patients worsened in the final score. Three patients improved 50% of the initial Nail Psoriasis Severity Index and only one had an improvement of 75%. Linear correlation showed a weak association between the improvement percentage in PASI and Nail Psoriasis Severity Index (r = 0.105, F = 2.12, p = 0.162). CONCLUSION: The method was easy and of rapid execution while potentially bringing information about changes in nail plate and matrix during treatment. The Nail Psoriasis Severity Index does not quantify the existing lesions and might not have the sensitivity to detect small changes.

FUNDAMENTOS: instrumentos de avaliação da psoríase ungueal têm sido publicados na literatura como meio de padronizar as avaliações antigamente subjetivas dos autores. OBJETIVOS: utilizar índice de Gravidade da Psoríase Ungueal ou Nail Psoriasis Severity Index para avaliação em pacientes portadores de psoríase em placas durante o tratamento com acitretina. MÉTODOS: trinta voluntários portadores de psoríase em placas foram selecionados para o estudo. Vinte deles ( 8 mulheres e 12 homens) completaram o estudo. A dose inicial da acitretina foi de 0,3mg/kg/dia por 30 dias sendo elevado posteriormente para 0,5mg/kg/dia. Utilizou-se a avaliação do Nail Psoriasis Severity Index na primeira avaliação, após 2 e 4 meses avaliando-se as unhas de ambas as mãos. RESULTADOS: lesões de unhas estavam presentes em todos os pacientes na primeira avaliação. A mediana inicial do Nail Psoriasis Severity Index foi de 20 e o escore final 20,5 (2,5% de piora ao final do estudo), sem diferença estatística significativa entre as três (X2=08084, GL=2, p=0,6657). Sete pacientes pioraram no escore final. Três pacientes obtiveram melhora de 50% do Nail Psoriasis Severity Index inicial em relação ao final e apenas 1 obteve melhora de 75 . A correlação linear simples mostrou fraca associação entre a porcentagem de melhora do PASI e do Nail Psoriasis Severity Index (r=0,105, F=2,12, p=0,l62). CONCLUSÕES: o método se mostrou de fácil e de rápida execução, podendo trazer informações sobre as alterações de lâmina e matriz de um modo global. O Nail Psoriasis Severity Index não quantifica as lesões existentes isoladamente, podendo não ter a sensibilidade de detectar pequenas alterações.

Diseño y evaluación in vivo de fórmulas para acné basadas en aceites esenciales de naranja (Citrus sinensis), albahaca (Ocimum basilicum L) y ácido acético / Effectiveness of antimicrobial formulations for acne based on orange (Citrus sinensis) and sweet basil (Ocimum basilicum L) essential oils

Introducción. Actualmente, la resistencia a los antimicrobianos de las cepas bacterianas involucradas en el desarrollo del acné es una realidad y se hace necesario buscar alternativas terapéuticas para su tratamiento. Objetivos. Diseñar fórmulas en gel a base de aceites esenciales y ácido acético, y evaluar su efectividad en pacientes voluntarios afectados por acné. Materiales y métodos. Se trata de un estudio experimental simple enmascarado de tres fórmulas en gel sobre cuatro grupos de siete pacientes. Los tratamientos antibacterianos (aceites esenciales), queratolíticos y mixtos (ácido acético), se aplicaron diariamente por espacio de ocho semanas. Se hicieron controles semanales para evaluar la evolución de los pacientes. Resultados. Todos los grupos reportaron mejoría (desaparición de las lesiones) de la condición del acné, la cual osciló entre 43 y 75 %, con leves efectos secundarios transitorios relacionados con la aplicación de los tratamientos utilizados. Conclusiones. Las fórmulas estudiadas mostraron ser estables química y físicamente durante la aplicación de los tratamientos, lo cual se demostró mediante análisis de cromatografía de gases, en la cual no se evidenció ningún cambio en los perfiles de composición de los aceites esenciales ni en el del ácido acético. Los resultados se catalogaron entre buenos y excelentes, en particular, el del ácido acético, que logró mejorías superiores al 75 %, dada su actividad mixta antiséptica y queratolítica. Los efectos secundarios (ardor y enrojecimiento) desaparecieron a los pocos minutos de realizada la aplicación y no impidieron el cumplimiento de los tratamientos.

Introduction. Currently, the antimicrobial resistance has developed in bacterial strains involved in the development of acne. Therefore, alternatives to antibiotic treatment have become necessary. Objectives. Gel formulations were designed based on essential oils and acetic acid, and their effectiveness was evaluated in patients affected by acne. Materials and methods. Masked simple experimental study of three gel formulations on 28 volunteer patients, separated in four groups of seven patients. Treatments were applied daily for eight weeks and consisted of (1) antibacterial (essential oils), (2) keratolytic medication (3) essential oils mixed with acetic acetic, and (4) kerolytic medication with acetic acid. Weekly checks were conducted to evaluate patient improvement. Results. All groups reported an improvement of the acne condition, which ranged between 43% and 75% clearance of lesions. Evidence of treatment disappeared within minutes, showing little discomfort or side effects after application. Conclusions. The essential oil formulations were chemically and physically stable during application of treatments. This was demonstrated by gas chromatography, where no evidence no change neither the composition profiles of essential oils nor in acetic acid. The results were ranked good to excellent, particularly for the acetic acid mixture, which achieved improvements of 75%. This appeared to be a result of their joint antiseptic and keratolytic activity. Side effects (burning and redness) disappeared within a few minutes of completing the application, therefore, did not interfere with adherence to treatment.

Pitiriasis rubra pilaris eritrodérmica: algo para no olvidar / Erythrodermic pityriasis rubra pilaris

La Pitiriasis Rubra Pilaris (PRP) es una dermatosis papuloescamosa crónica, de etiología desconocida. Se caracteriza por la presencia de pápulas foliculares hiperqueratósicas que coalescen formando placas eritematoescamosas, dejando islotes de piel sana entre las lesiones. Puede llegar a una eritrodermia. Se clasifica en base a la edad de presentación, características morfológicas, evolución y pronóstico. Existen múltiples opciones de tratamiento descritas en la literatura, siendo los retinoides sistémicos el tratamiento de primera línea en estos pacientes. Presentamos dos casos de pacientes con PRP eritrodérmica tratados exitosamente con Acitretín y revisión de la literatura a la fecha.

Pityriasis Rubra Pilaris is a chronic inflammatory dermatosis of unknown etiology, characterized by the presence of multiple follicular papules that coalesce into large erythematous or salmon colored plaques with islands of non-involved skin between them. It can eventually evolve into erythroderma. Descriptions and therapeutic experiences are mainly based on case reports. Today retinoids have become de first line treatment in these patients. We present two cases of erythrodermic PRP treated successfully with Acitretin and an updated review of the literature.

Líquen plano hipertrófico disseminado: relevante resposta à acitretina / Disseminated hypertrophic lichen planus: relevant response to acitretin

O líquen plano hipertrófico é uma variante do líquen plano, com pronunciada hiperplasia epidérmica em resposta à coçadura persistente. Clinicamente, caracterizam-se por placas hiperceratósicas, simétricas, de coloração cinza-violácea, com predileção pela região pré-tibial. O prurido intenso, a refratariedade aos tratamentos convencionais e a possibilidade de associação de um carcinoma epidermoide às lesões de longa duração impõem um tratamento eficaz. Os corticoides são considerados o tratamento de primeira linha e podem ser aplicados topicamente ou empregados de forma sistêmica. Outras modalidades terapêuticas propostas são a fototerapia com UVB-NB ou PUVA, imunossupressores e retinoides sistêmicos, com destaque para a acitretina. Relatamos um caso com apresentação clínica exuberante e excelente resposta à acitretin, totalizando um seguimento de nove meses.

Hyperthrofic lichen planus is considered a variant of lichen planus with marked epidermal hyperplasia in response to persistent itch. It is clinically, characterized by symmetric hyperkeratotic plaques, of purplish-grey color, often located in the pretibial region. Intense pruritus, refractoriness to conventional treatments and the possibility of association of the long-term injuries with squamous cell carcinoma requires an effective treatment. The first-line treatment is corticosteroids which can be applied either topically or systemically. Other therapeutic modalities proposed are: NB-UVB phototherapy or PUVA, immunosuppressive drugs and systemic retinoids, notably acitretin. We report a case with exuberant clinical presentation of hyperthrofic lichen planus with excellent response to acitretin after nine months of treatment.

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

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

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