Evaluation of plasma and urinary levels of vascular endothelial growth factor and matrix metalloproteinase-9 in patients with infantile hemangioma.

BACKGROUND: The pathogenesis of infantile hemangioma (IH) is not fully understood. It has been suggested that angiogenic factors increase in the proliferative stage, decreasing subsequently in the regression phase. OBJECTIVES: To evaluate vascular endothelial growth factor (VEGF) and matrix metalloproteinase 9 (MMP-9) levels, according to infantile hemangioma (IH) growth stages and size, and to compare these levels in patients with IH and control subjects. METHODS: This cross-sectional study included 68 patients with IH and 25 control subjects. Plasma and urinary MMP-9 and VEGF levels were evaluated during proliferative and regression phases. These levels were correlated with tumor size measured by ultrasonography. Nonparametric tests were performed. RESULTS: Among 68 patients with IH, 55 (81%) were female. Age ranged from 1 to 40 months (median 7.0 months). There was no difference in plasma and urinary levels of VEGF and MMP-9 between patients and control subjects. There were no significant differences in these levels between IH patients younger or older than 12 months of age, as a cutoff between proliferative and involution phases. No significant correlation was observed between tumor size and levels of the markers (R < 0.20 and P > 0.05 for all comparisons). CONCLUSION: In our large sample, levels of VEGF and MMP-9 did not reflect the characteristic increased angiogenesis in patients with IH when compared to healthy subjects. In addition, these markers were not increased in the proliferative stage of the IH and did not correlate with tumor size.

PHACE syndrome: clinical manifestations, diagnostic criteria, and management.

Infantile hemangioma can be linked to other organ malformations. In 1996, PHACE syndrome was first defined as the association of large and segmental infantile hemangioma, usually on the face, head, or cervical region, with malformations of the posterior fossa of the brain, arterial anomalies of the central nervous system, coarctation of the aorta, cardiac defects, and ocular abnormalities. Over 300 cases of PHACE syndrome have been reported, and it is cconsidered one of the most common neurocutaneous vascular disorders in childhood. Knowledge of the features and locations of lesions that imply a greater risk of systemic involvement is crucial for the diagnosis and proper management of PHACE syndrome patients. This review highlights the diagnostic criteria for PHACE syndrome, the imaging workup for extracutaneous involvement, the treatment of infantile hemangioma, and the importance of a multidisciplinary approach in the management of these patients.

PHACE syndrome: clinical manifestations, diagnostic criteria, and management

Abstract: Infantile hemangioma can be linked to other organ malformations. In 1996, PHACE syndrome was first defined as the association of large and segmental infantile hemangioma, usually on the face, head, or cervical region, with malformations of the posterior fossa of the brain, arterial anomalies of the central nervous system, coarctation of the aorta, cardiac defects, and ocular abnormalities. Over 300 cases of PHACE syndrome have been reported, and it is cconsidered one of the most common neurocutaneous vascular disorders in childhood. Knowledge of the features and locations of lesions that imply a greater risk of systemic involvement is crucial for the diagnosis and proper management of PHACE syndrome patients. This review highlights the diagnostic criteria for PHACE syndrome, the imaging workup for extracutaneous involvement, the treatment of infantile hemangioma, and the importance of a multidisciplinary approach in the management of these patients.

Infantile hemangioma: pathogenesis and mechanisms of action of propranolol.

Infantile hemangioma (IH) is the most common benign tumor of childhood, with a prevalence of 4 % to 10 %. It is characterized by a proliferative rapid growth phase, which starts after a few weeks of life, followed by a slow regression phase. In IH cases that are potentially disfiguring or life-threatening (10 % to 15 % of all cases), systemic therapy should be promptly initiated. Data source The present study reviews published scientific articles available in reliable electronic databases. Selected were all studies that evaluated the pathogenesis of IH and the mechanisms of action of propranolol. Conclusions The pathogenesis of IH has not been fully elucidated. Studies show that, in the proliferative phase of IH, there is an imbalance of angiogenic factors and an increase in the levels of vascular endothelial growth factor and matrix metalloproteinases 2 and 9. In the regression phase, the levels of these factors decrease, whereas those of antiangiogenic factors, including tissue inhibitors of matrix metalloproteinases, increase. Since 2008, propranolol has become the drug of choice in the treatment of IH, targeting vascular tone, angiogenesis, and apoptosis. Current insights into the pathogenesis of IH allow for the development of new therapeutic strategies.

Estudo clínico para avaliar a eficácia e segurança de um hidratante ativo reparador de barreira como auxiliar no tratamento de dermatite atópica em crianças / Clinical evaluation of the efficacy and safety of an active moisturizer-barrier repairer as an adjuvant treatment in atopic dermatitis in children

Introdução: O uso de hidratantes melhora a função de barreira cutânea e pode ser útil na dermatite atópica. Objetivos: Avaliar eficácia e segurança de hidratante ativo reparador de barreira à base de glicerina, erythritol, Imperata cilindrica e homarine como tratamento auxiliar da dermatite atópica. Métodos: Aplicação de hidratante em crianças com dermatite atópica de leve a moderada durante um mês, com avaliações subjetivas e objetivas após 15 e 30 dias. Resultados: Foram analisados os dados de 35 crianças, de um a dez anos, quanto a: alterações do sono, ressecamento, descamação, prurido e maciez da pele, que tiveram redução significativa da visita inicial em relação à final. A avaliação objetiva do índice de gravidade Scorad (score on atopic dermatitis) revelou redução de 25.27 para 9.30 (p < 0.0001). Houve redução no uso de dexclorfeniramina de 25.71% no 15o dia para 5.72% no 30o dia (p = 0.0233) e de 34.29% no 15o dia para 5.72% no 30o dia com hidrocortisona (p = 0.0075). Conclusões: O uso do hidratante durante 30 dias melhorou os sintomas analisados, com significativa diminuição do índice de gravidade e menor necessidade de anti-histamínicos e corticosteroides tópicos. A utilização de hidratante adequado foi eficaz e segura como auxiliar para crianças com dermatite atópica.

Introduction: The use of moisturizers improves the skin barrier's function and might be useful in atopic dermatitis. Objectives: To assess the efficacy and safety of an active moisturizer-barrier repairer based on glycerin, erythritol, Imperata cylindrica and homarine as an adjuvant treatment in atopic dermatitis. Methods: Application of the moisturizer in children with mild to moderate atopic dermatitis for 1 month, with subjective and objective evaluations after 15 and 30 days. Results: Data from 35 children ­ 20 girls (57.15%), 15 boys (42.86%) / 26 Caucasians (74.29%), 6 mulattos (17.14%), 3 dark skinned (8.57%) ­ aged 1 to 10 years (mean age = 5.6) were analyzed. The values of the variables sleeping disorders, dryness, desquamation, pruritus and softness of the skin had significantly decreased on the 30th day of treatment as compared to the baseline. The objective evaluation of the severity using the Scoring Atopic Dermatitis severity index revealed a decrease to 9.30 from 25.27 (p <0.0001). There was a reduction in the use of dexchlorpheniramine and hydrocortisone to 5.72% each on the 30th day, from 25.71% (p = 0.0233) and 34.29% (p = 0.0075) on the 15th day, respectively. Conclusions: The use of the moisturizer for 30 days improved the analyzed symptoms with a significant reduction in the Scoring Atopic Dermatitis severity index with decreased need for antihistamines and topical corticosteroids. The use of a suitable moisturizer was effective and safe as an adjuvant treatment for children with atopic dermatitis.

Ultrasonography as an objective tool for assessment of infantile hemangioma treatment with propranolol.

BACKGROUND: Infantile hemangiomas (IHs) are the most common benign vascular tumors of childhood. Propranolol is an effective drug in treating IH. A reliable and complementary instrument is necessary to evaluate IH response to propranolol in addition to clinical and photographic assessments. Ultrasonography is a simple and non-invasive technique that enables precise measurements of tumor size and contributes to objective follow-up. OBJECTIVE: To demonstrate the use of serial ultrasonography as an adjunctive tool for assessment of IH treatment with propranolol. PATIENTS AND METHODS: A retrospective study of 19 patients with IH treated with propranolol was conducted from January 2009 to March 2014. Data of individual IH volume at the beginning and at least 6 months after the onset of treatment and overall volume reduction by ultrasonographic measurement were obtained. RESULTS: We observed a statistically significant IH volume reduction of approximately 0.51 cm3 . This volume corresponds to an average reduction of 47% in the final volume compared with the initial volume. CONCLUSION: Ultrasonographic measurements contribute to demonstrate tumor regression and IH response to propranolol. Thus, ultrasonography is an important instrument to guide therapeutic strategies.

Propranolol for treatment of infantile hemangiomas.

Infantile hemangioma is the most common vascular tumor in early childhood. Propranolol has been successfully used recently in a limited number of children with Infantile hemangioma. We present 6 cases of Infantile hemangioma, at a single dermatological center, which responded to oral propranolol with good results.

Propranolol for treatment of infantile hemangiomas / Tratamento de hemangiomas infantis com propranolol

Infantile hemangioma is the most common vascular tumor in early childhood. Propranolol has been successfully used recently in a limited number of children with Infantile hemangioma. We present 6 cases of Infantile hemangioma, at a single dermatological center, which responded to oral propranolol with good results.

O hemangioma da infância é o tumor vascular mais comum nessa faixa etária. Mais recentemente, propranolol oral tem sido usado com sucesso em um número limitado de crianças com hemangioma da infância. Nós apresentamos 6 casos de hemangioma da infância, provenientes de um único centro dermatológico, que apresentaram boa resposta ao tratamento com propranolol oral.

O'Brien's actinic granuloma: an unusually extensive presentation.

O'Brien's actinic granuloma is a rare skin disease. Controversy continues over whether it should be considered a specific condition or a form of granuloma annulare located in sun-exposed areas. Its pathogenesis is unknown; however, the most widely accepted hypothesis suggests that solar radiation is the triggering factor. This paper describes the case of a 78-year old, fair-skinned male, who presented with a 10-year history of an infiltrate of annular erythematous papules on his forehead and left malar region. The diagnosis of O'Brien actinic granuloma was established from histopathology, since the clinical condition of the patient was extensive, unlike cases reported in the literature.

Granuloma actínico de O'Brien: apresentação clínica exuberante / O'Brien's actinic granuloma: an unusually extensive presentation

O granuloma actínico de O'Brien é uma doença cutânea rara. Há muitas controvérsias na sua caracterização, podendo representar uma entidade nosológica distinta ou uma forma de granuloma anular em áreas expostas. A patogênese é desconhecida; no entanto, acredita-se que a radiação solar seja um fator desencadeante. Os autores relatam o caso de um paciente de 78 anos, branco, que apre sentava há 10 anos pápulas eritematosas e infiltradas na fronte e hemiface à esquerda. O diagnóstico de granuloma actínico de O'Brien foi estabelecido a partir do exame histopatológico, uma vez que o quadro clínico exuberante apresentado pelo paciente diferia do de casos anteriormente observados.

O'Brien's actinic granuloma is a rare skin disease. Controversy continues over whether it should be considered a specific condition or a form of granuloma annulare located in sun-exposed areas. Its pathogenesis is unknown; however, the most widely accepted hypothesis suggests that solar radiation is the triggering factor. This paper describes the case of a 78-year old, fair-skinned male, who presented with a 10-year history of an infiltrate of annular erythematous papules on his forehead and left malar region. The diagnosis of O'Brien actinic granuloma was established from histopathology, since the clinical condition of the patient was extensive, unlike cases reported in the literature.

Frequency of occupational contact dermatitis in an ambulatory of dermatologic allergy.

BACKGROUND: Occupational contact dermatitis (OCD) corresponds to 80% of the cases of skin diseases related to work. OBJECTIVES: To determine: the frequency of OCD in an ambulatory of allergy not specific for occupational dermatosis; the profile of these patients according to age, gender, color, profession, atopic history, duration and localization of the dermatosis; the diagnosed types of contact dermatitis; the main allergens and to compare the frequency of the main antigens with the results obtained from patients examined in the same period of time, without DCO. METHODS: During the three-year period from 2003 to 2006, 630 patients were examined and submitted to a battery of standardized contact patch tests and those who were diagnosed with OCD were selected. RESULTS: Sixty-nine out of the 630 patients were diagnosed with OCD. The average age was 44.5 years. Forty-eight patients (70%of the cases) presented the disease on the hands. The most prevalent activities of patients suffering from the disease were household tasks (39%) and building construction (33%). The allergic contact dermatitis (ACD) occurred in 48 cases which represent 70% of the total number of cases and irritant contact dermatitis in 21 cases corresponding to 30% of the total number of cases. The main allergens were potassium dichromate 28 (41%), nickel sulfate 16 (23%) and carba-mix 16 (23%). CONCLUSION: OCD was present in 10.9% of the patients. Concerning age the group in which the disease was more commonly found corresponded to the most productive group of the population. ACD corresponded to 48 cases, probably influenced by the long period of evolution of the dermatosis. Metal and rubber chemicals were the commonest allergens.

Frequência da dermatite de contato ocupacional em ambulatório de alergia dermatológica / Frequency of occupational contact dermatitis in an ambulatory of dermatologic allergy

FUNDAMENTOS: A dermatite de contato ocupacional (DCO) corresponde a 80 por cento dos casos de dermatoses relacionadas ao trabalho. OBJETIVOS: Determinar: a frequência de DCO em pacientes atendidos em ambulatório de alergia, não específico de dermatose ocupacional; o perfil dos pacientes de acordo com idade, sexo, cor, profissão, antecedentes atópicos, duração e localização da dermatose; os tipos de dermatite de contato; os principais alérgenos; e comparar as frequências dos principais sensibilizantes com os resultados obtidos em pacientes atendidos no mesmo período, sem DCO. MÉTODOS: Durante o período 2003-2006, 630 pacientes foram atendidos e submetidos à bateria padrão de testes de contato. Selecionaram-se aqueles com DCO. RESULTADOS: Sessenta e nove (10,9 por cento) pacientes apresentaram DCO. A média de idade foi de 44,5 anos. As mãos foram acometidas em 48 (70 por cento) casos. As profissões prevalentes foram do lar (27,39 por cento) e construção civil (23; 33,5 por cento). Dermatite alérgica de contato (DAC) ocorreu em 48 (70 por cento) casos, e dermatite irritativa de contato (DIC), em 21 (30 por cento). Os principais sensibilizantes foram o bicromato de potássio (28; 41 por cento), sulfato de níquel (16; 23 por cento) e carba-mix (16; 23 por cento). CONCLUSÕES: A DCO foi diagnosticada em 10,9 por cento dos pacientes, sendo mais comum na faixa etária produtiva da população. DAC esteve presente em 48 casos, provavelmente influenciada pelo longo tempo de evolução da dermatose. Os metais e os componentes da borracha foram os principais sensibilizantes.

BACKGROUND: Occupational contact dermatitis (OCD) corresponds to 80 percent of the cases of skin diseases related to work. OBJECTIVES: To determine: the frequency of OCD in an ambulatory of allergy not specific for occupational dermatosis; the profile of these patients according to age, gender, color, profession, atopic history, duration and localization of the dermatosis; the diagnosed types of contact dermatitis; the main allergens and to compare the frequency of the main antigens with the results obtained from patients examined in the same period of time, without DCO. METHODS: During the three-year period from 2003 to 2006, 630 patients were examined and submitted to a battery of standardized contact patch tests and those who were diagnosed with OCD were selected. RESULTS: Sixty-nine out of the 630 patients were diagnosed with OCD. The average age was 44.5 years. Forty-eight patients (70 percentof the cases) presented the disease on the hands. The most prevalent activities of patients suffering from the disease were household tasks (39 percent) and building construction (33 percent). The allergic contact dermatitis (ACD) occurred in 48 cases which represent 70 percent of the total number of cases and irritant contact dermatitis in 21 cases corresponding to 30 percent of the total number of cases. The main allergens were potassium dichromate 28 (41 percent), nickel sulfate 16 (23 percent) and carba-mix 16 (23 percent). CONCLUSION: OCD was present in 10.9 percent of the patients. Concerning age the group in which the disease was more commonly found corresponded to the most productive group of the population. ACD corresponded to 48 cases, probably influenced by the long period of evolution of the dermatosis. Metal and rubber chemicals were the commonest allergens.

The role of glass as a barrier against the transmission of ultraviolet radiation: an experimental study.

BACKGROUND/PURPOSE: Excessive exposure of the skin to sunlight may cause many symptoms and skin cancer. The aim was to measure the transmission of ultraviolet (UV) A and UVB radiation through glasses of different types, according to the distance from the light source. METHODS: The baseline radiation from UVA and UVB sources was measured at different distances from the photometers. Next, the radiation from the same sources was measured at the same distances, but transmitted by different types of glass. The baseline values were compared with the results after protection using glass. RESULTS: Laminated glass totally blocked UVA radiation, while smooth ordinary glass transmitted the highest dose (74.3%). Greater thicknesses of glass implied less radiation transmitted, but without a significant difference. Green glass totally blocked UVA radiation, while blue glass transmitted the highest dose of radiation (56.8%). The presence of a sunlight control film totally blocked UVA radiation. All glasses totally blocked UVB radiation. CONCLUSION: The main characteristics of glass that make it a photoprotective agent are its type (especially laminated glass) and color (especially green), which give rise to good performance by this material as a barrier against the transmission of radiation.

Você conhece esta síndrome? / Do you know this syndrome?

A osteodistrofia hereditária de Albright é caracterizada por calcificações cutâneas, obesidade, baixa estatura, braquidactilia associada ao pseudo-hipoparatireoidismo do tipo IA entre outras alterações hormonais como hipotireoidismo e hipogonadismo. O diagnóstico é baseado no quadro clínico associado aos achados de hipocalcemia e níveis elevados de hormônio da paratireóide. Os autores relatam caso em que a avaliação dermatológica foi de grande contribuição para o diagnóstico.

Albright hereditary osteodystrophy is characterized by subcutaneous calcification, obesity, short stature, brachydactyly and pseudohypoparathyroidism type IA. Hypothyroidism and hypogonadism may be present. The diagnosis is based on clinical characteristics associated with hypocalcemia and high levels of parathyroid hormone. The authors report a case in which the dermatological evaluation contributed to diagnosis.