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)

Therapeutic response and safety of the topical, sequential use of antiseptic, keratolytic, and pentamidine creams (3-PACK) on Leishmania (Viannia) braziliensis-infected mice

BACKGROUND Topical treatment of New World cutaneous leishmaniasis can be affected by bacterial coinfection, hyperkeratosis, and transdermal drug delivery. OBJECTIVE The aim of this work was to evaluate the therapeutic response and safety of the topical, sequential use of antiseptic, keratolytic, and pentamidine isethionate (PMD) creams (3-PACK kit) on CL-infected BALB/c mice. METHODS A 0.5% chlorhexidine solution (CGH), 10% salicylic acid (SA), and 3% or 6% PMD were used as antiseptic, keratolytic, and antileishmanial drugs, respectively. During the first seven days, antiseptic, followed by 10% SA gel and PMD cream, were applied topically. Subsequently, treatment was performed only with the antiseptic and PMD creams. Skin irritation, reduction of lesion size (mm2), and parasitic load were observed until 30 days of treatment were completed. FINDINGS The 3-PACK treatment using 6% PMD induced a complete lesion reduction in 3/6 mice and a partial reduction in 1/6 mice, with no parasites observed. In contrast, CGH and SA alone, along with the vehicle, were not effective (p < 0.05). Moderate to severe erythema was observed at the application site. MAIN CONCLUSION The topical 3-PACK using 6% PMD was 67% effective in the treatment of CL by Leishmania (Viannia) braziliensis. Currently, work is ongoing to improve PMD isethionate formulation and to determine a dose-response.

A Case of Elephantiasis Nostras Verrucosa Treated with Oral Acitretin / 대한피부과학회지

Elephantiasis nostras verrucosa (ENV) is an uncommon condition caused by repeated inflammation and lymphatic obstruction. It occurs mainly in the lower extremities and is characterized by skin changes, including hyperkeratotic mossy papules and plaques, non-pitting edema, and cobblestone-like appearances. ENV can be diagnosed based on its typical clinical manifestations (pseudoepitheliomatous hyperplasia with enlarged lymphatic spaces, fibrous tissue proliferation, and chronic inflammation) and by skin biopsy. Although ENV is difficult to treat, reduction of lymphedema and use of keratolytic agents may be helpful. To our knowledge, only three cases of ENV have been reported. However, the case of ENV treated with oral acitretin has not been reported in domestic literatures. Therefore, we report the case of a 45-year-old woman who developed ENV. She had a past history of cellulitis and was successfully treated with oral acitretin.

Case for diagnosis. Linear Darier's disease

Abstract: We present a different and rare manifestation of Darier's disease, namely linear Darier's disease. Only a few cases have been described in the literature. The case report is a male patient, 60 years old, presenting brown to red papules and plaques with hyperkeratosis distributed on the abdomen, following Blaschko's lines, with 6 years' evolution. It was a difficult diagnosis until the dermatological workup and biopsy.

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.

Combination of topical agents and oxybutynin as a therapeutic modality for patients with both osmidrosis and hyperhidrosis / Uso combinado de agentes tópicos e oxibutinina para tratamento de pacientes com osmidrose e hiper-hidrose

Summary Introduction: The association of osmidrosis and hyperhidrosis often causes emotional and social problems that may impair the patients' quality of life. The purpose of our study was to analyze the therapeutic results of oxybutynin and topical agents in 89 patients with both osmidrosis and hyperhidrosis. Method: We conducted an observational study at two specialized centers of hyperhidrosis between April 2007 and August 2013. Eighty-nine (89) patients with both osmidrosis and hyperhidrosis were treated with oxybutynin and topical agents. Patients were evaluated before treatment and at 3 and 6 weeks after treatment started, by using the Quality of Life Questionnaire and the Sweating Evolution Scale. Results: Before treatment, 98% of the patients presented with poor or very poor quality of life. After six weeks of treatment, 70% stated their quality of life as being slightly better or much better (p<0.001) and nearly 70% of the patients experienced a moderate or great improvement in sweating and malodor. Improvement in osmidrosis was significantly greater when the axillary region was the first most disturbing site of hyperhidrosis. Conclusion: There was a significant improvement in quality of life and a reduction in sweating and malodor after six weeks of treatment with topical agents and oxybutynin in patients with both hyperhidrosis and osmidrosis. Therefore, clinical treatment should be considered before invasive techniques.

Resumo Introdução: A associação entre osmidrose e hiper-hidrose com frequência causa problemas emocionais e sociais que podem deteriorar a qualidade de vida dos pacientes. O objetivo deste estudo foi analisar os resultados terapêuticos do uso de oxibutinina associada a agentes tópicos em 89 pacientes com osmidrose e hiper-hidrose. Método: Nós conduzimos um estudo observacional em dois centros especializados em hiper-hidrose entre abril de 2007 e agosto de 2013. Oitenta e nove (89) pacientes com osmidrose associada a hiper-hidrose foram tratados com oxibutinina e agentes tópicos. Os pacientes foram avaliados antes do tratamento e após 3 e 6 semanas do início do tratamento, por meio do Questionário de Qualidade de Vida e da Escala de Evolução da Sudorese. Resultados: Antes do tratamento, 98% dos pacientes apresentavam qualidade de vida ruim ou muito ruim. Após seis semanas de tratamento, 70% classificou sua qualidade de vida como sendo pouco ou muito melhor (p<0.001) e aproximadamente 70% dos pacientes relataram melhora moderada ou grande de sudorese e odor. Houve melhora significativamente maior da osmidrose quando a região axilar era o sítio em que a hiper-hidrose mais incomodava. Conclusão: Houve melhora significativa da qualidade de vida e uma redução da sudorese e do odor após seis semanas de tratamento com agentes tópicos e oxibutinina em pacientes com hiper-hidrose associada a osmidrose. Dessa maneira, a terapia clínica deve ser considerada antes de técnicas invasivas.

Tretinoin peel: a critical view

Abstract The tretinoin peel, also known as retinoic acid peel, is a superficial peeling often performed in dermatological clinics in Brazil. The first study on this was published in 2001, by Cuce et al., as a treatment option for melasma. Since then, other studies have reported its applicability with reasonable methodology, although without a consistent scientific background and consensus. Topical tretinoin is used for the treatment of various dermatoses such as acne, melasma, scars, skin aging and non-melanoma skin cancer. The identification of retinoids cellular receptors was reported in 1987, but a direct cause-effect relation has not been established. This article reviews studies evaluating the use of topical tretinoin as agent for superficial chemical peel. Most of them have shown benefits in the treatment of melasma and skin aging. A better quality methodology in the study design, considering indication and intervention is indispensable regarding concentration, vehicle and treatment regimen (interval and number of applications). Additionally, more controlled and randomized studies comparing the treatment with tretinoin cream versus its use as a peeling agent, mainly for melasma and photoaging, are necessary.

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.

Effect of pharmacological intervention on MIP-1α, MIP-1β and MCP-1 expression in patients with psoriasis vulgaris

OBJECTIVE@#To detect the expression level of macrophage inflammatory protein-1 (MIP-1)α, MIP-1β and monocyte chemoattractant protein-1 (MCP-1) in with psoriasis vulgaris and explore the role in the pathogenesis of psoriasis vulgaris.@*METHODS@#The level of MIP-1α, MIP-1β and MCP-1 in peripheral blood from 50 patients with psoriasis vulgaris and 50 normal controls were measured by enzyme linked immunosorbent assay. The correlation with psoriasis area and severity index (PASI) was analyzed. The level of MIP-1α, MIP-1β and MCP-1 was compared between psoriasis vulgaris patients at active stage and resting stage. And the change in MIP-1α, MIP-1β and MCP-1 before and after therapy was also observed.@*RESULTS@#The content of MIP-1α, MIP-1β and MCP-1 in patients with psoriasis vulgaris was (1342.78 ± 210.30), (175.28 ± 28.18) and (266.86 ± 32.75) ng/L, respectively, significantly higher than those in control group (P<0.05). The expression level of MIP-1α, MIP-1β and MCP-1 in peripheral blood of patients with psoriasis vulgaris was positively correlated with PASI (P<0.01). After acitretin therapy, expression level of MIP-1α, MIP-1β and MCP-1 in peripheral blood of patients with psoriasis vulgaris was significantly decreased.@*CONCLUSIONS@#Chemokine factor MIP-1α, MIP-1β and MCP-1 may be involved in the pathogenesis of psoriasis vulgaris.

Randomized clinical efficacy of superficial peeling with 85% lactic acid versus 70% glycolic acid / Estudo randomizado da eficacia clinica dos peelings superficiais de acido lactico 85% versus acido glicolico 70%

BACKGROUND: Peeling is a procedure which aims to accelerate the process of skin exfoliation. OBJECTIVES: Development of formulations containing lactic acid at 85% or glycolic acid at 70% and the evaluation of these formulations on clinical efficacy in reduction of fine wrinkles. METHODS: Preliminary stability tests were carried out and an in vivo study was performed with three groups with 9 representatives each. One was the control group, which used only sunscreen; another one used lactic acid+sunscreen, and the last group used acid glycolic+sunscreen. Clinical efficacy was assessed with a CCD color microscope, through the digitization of images before and after treatment. The applications were carried out by a dermatologist, once a mont h every 30 days, during 3 months. The area with wrinkles was calculated by planimetry point counting, in accordance with Mandarin-de-Lacerda. RESULTS: The formulations were stable in the visual and Ph evaluation. There was no improvement in the control group; for lactic acid, there was significant improvement after the second peeling application on the outer lateral area of the right eye and after the third application on the outer lateral area of the left eye. For the glycolic acid group, there was significant improvement in the outer lateral area of the left eye after the first application, and of the right eye region, after three applications. The formulations used must be kept under refrigeration and should be manipulated every 30 days. CONCLUSIONS: Both peelings were effective in reducing fine wrinkles of the outer lateral eye area after three applications (p≤0.05%). It was observed that peeling efficacy in the external-lateral region of one eye might be different compared with that in skin of the external-lateral region of the other eye, relative to the speed of skin improvement. .

FUNDAMENTOS: Peeling visa a acelerar o processo de esfoliação da pele. OBJETIVOS: Desenvolver formulações contendo ácido láctico a 85% ou ácido glicólico a 70% e avaliar sua eficácia clínica na redução de rugas finas. MÉTODOS: Testes preliminares foram efetuados e estudo in vivo foi realizado em três grupos com nove representantes cada, separados de forma randomizada. Um grupo foi controle, utilizando apenas fotoprotetor; outro utilizou ácido láctico e fotoprotetor; o último usou ácido glicólico e fotoprotetor. Para eficácia clínica, empregou-se microscópio CCD color, digitalizando-se as imagens do pré e do pós-tratamento. As aplicações foram realizadas por médica dermatologista uma vez por mês, a cada 30 dias, durante três meses. A área com traços de ruga foi calculada pela planimetria por contagem de pontos. RESULTADOS: As formulações foram estáveis na avaliação visual e de pH. Não houve melhora no grupo controle; para o grupo do ácido láctico, houve melhora significativa após a segunda aplicação do peeling na região lateral externa do olho direito e após a terceira aplicação na região lateral externa olho esquerdo. Para o grupo do ácido glicólico, houve melhora significativa na região lateral externa olho esquerdo após a primeira aplicação e, depois de três aplicações, na região lateral externa do olho direito. As formulações magistrais empregadas no estudo devem ser mantidas sob refrigeração e manipuladas a cada 30 dias. CONCLUSÕES: Tanto o peeling de ácido láctico quanto o de ácido glicólico foram eficazes na diminuição de rugas finas da região lateral externa dos olhos após ...