ABSTRACT Objective: The aim of this study was to elaborate a specific protocol for the assessment and early identification of skin lesions in pediatric patients with Fanconi anemia undergoing hematopoietic stem cell transplantation. Methods: This is a longitudinal, retrospective, and descriptive study. The medical records of 136 pediatric patients with Fanconi anemia who underwent hematopoietic stem cell transplantation between 2008 and 2018 at the Clinical Hospital of the Federal University of Paraná were reviewed. A specific protocol was created for data collection, which included age, sex, skin color, age at diagnosis of Fanconi anemia, transplantation data, family history of consanguinity, and pre- and post-transplant complications. In addition, the data included the presence of graft-versus-host disease of the skin and other organs, its classification, type of lesion, location, and also skin lesions not related to graft-versus-host disease. Results: Among the skin manifestations in pre-transplant period, café-au-lait spots stood out (32.4%). At least one organ was affected by graft-versus-host disease in 55.1% of patients; the most common involvement being the mouth, followed by the skin. Rash and erythema were the most frequently observed cutaneous manifestations of graft-versus-host disease. Conclusion: A high prevalence of cutaneous manifestations of the disease was observed, as well as cutaneous manifestations of graft-versus-host disease. The protocol developed gathers relevant and standardized information for the follow-up of patients with Fanconi anemia undergoing hematopoietic stem cell transplantation, ensuring greater reliability of the information, and its implementation will allow the prospective evaluation of patients.

RESUMO Objetivo: Elaborar um protocolo específico para a avaliação e identificação precoces de lesões de pele em pacientes pediátricos com anemia falciforme submetidos ao transplante de células-tronco hematopoiéticas. Métodos: Estudo longitudinal, retrospectivo e descritivo. Foram revisados os prontuários dos pacientes pediátricos com anemia de Fanconi submetidos a transplante de células-tronco hematopoiéticas entre os anos de 2008 e 2018 no Hospital de Clínicas da Universidade Federal do Paraná, totalizando 136 pacientes. Foi criado um protocolo específico para a coleta de dados, que incluiu: idade, sexo, cor, idade ao diagnóstico da anemia de Fanconi, dados do transplante, história familiar de consanguinidade e complicações pré e pós-transplante. Além disso, foram verificados a presença de doença do enxerto contra o hospedeiro da pele e de outros órgãos, sua classificação, tipo de lesão, localização e, também, lesões de pele não relacionadas à doença. Resultados: Entre as manifestações de pele no período pré-transplante, destacaram-se as manchas café com leite (32,4%). Pelo menos um órgão foi afetado pela doença do enxerto contra o hospedeiro em 55,1% dos pacientes, sendo o acometimento mais comum o de boca, seguido pelo de pele. Exantema e eritema foram as manifestações cutâneas mais frequentemente observadas. Conclusões: Observou-se alta prevalência de manifestações cutâneas próprias da doença, bem como de manifestações cutâneas de doença do enxerto contra o hospedeiro. O protocolo elaborado reúne informações relevantes e padronizadas para o acompanhamento dos pacientes com anemia de Fanconi submetidos ao transplante, garantindo maior confiabilidade das informações, e sua implementação permitirá a avaliação prospectiva dos pacientes.

Abstract Objective: Evaluate the effectiveness of a children's soap with physiological pH in maintaining cutaneous pH and moisture of the newborn (NB)’s skin after the first bath. Methods: Randomized, controlled and double-blind clinical trial in a rooming-in of a tertiary maternity hospital in southern Brazil with 204 newborns > 34 gestational weeks. Gestational and obstetric history was evaluated, and newborns were randomized into two groups according to the product applied in the bath: the control group (CG), which used common liquid soap with pH 7.0 and experimental group (EG), which used children's liquid soap with pH 5.8. Evaluation was made immediately before and after bath with skin pH measurement, corneometry and clinical parameters (erythema, scaling and moisture), on the forehead, abdomen and thigh. Results: There was no difference between groups regarding gestational, obstetric and family history (p > 0.05). In CG, skin pH increased in the abdomen and thigh (p < 0.05). In EG there was an improvement in clinical parameters after bathing with: increased moisture, less erythema and less scaling (p < 0.05). On the forehead, there was a significant increase in pH after bathing (p < 0.001) similar in both groups, although no use of soap. There was no difference in corneometry between groups after bathing. Conclusions: Children's liquid soap with physiological pH maintained the acidic skin pH and moisture of the newborn's skin after the first bath, which reinforces the importance of using products with physiological pH in the hygiene of newborns. Registration number RBR-9ky84vd.

Abstract: Background: The greatest sun exposure occurs until adolescence and this is the only factor involved in photocarcinogenesis subject to modification with photoprotection. Objective: The objective of this study was to evaluate the habits and knowledge of adolescents regarding photoprotection. Method: Cross-sectional study that included 512 students from primary and high school. Data were collected by questionnaires with questions about habits and knowledge on sun protection. For data analysis, Pearson's Chi-square test and Yates test were applied, with 5% level of significance. Results: We evaluated 512 students, with a mean age of 14 years old. The teenagers agreed that ultraviolet rays cause skin cancer and aging (96% and 70%, respectively). Knowledge about the occurrence of burns even on cloudy days was recorded in 68% of cases, as well as the need for photoprotection for sports in 72%. The source of information about sun protection were by parents in 55%. In 70% was observed previous occurrence of sunburn, the daily use of sunscreens was found in 15% and 67% used only at the beach. of sunscreens with SPF higher than 30 was reported by 70% of students and 57% reported going to the beach between 10h and 16h. In 68% of cases it was registered insufficient sun protection, attributed, in 57% of the time to forgetfulness. Study limitations: The sample refers to two schools of Curitiba (PR), Brazil. Conclusion: Teenagers know the risks of sun exposure, but they do not adopt the practices for adequate protection.

Resumo Objetivos: Avaliar o pH dos sabonetes infantis, antibacterianos e sindets (syntetic detergents) habitualmente usados em crianças, bem como verificar se há no rótulo desses produtos informação sobre seu pH. Métodos: Estudo observacional, analítico, transversal e quantitativo, que incluiu 90 sabonetes nas apresentações em barra e líquida, 67 infantis (grupo 1), 17 antibacterianos (grupo 2) e 6 sindets (grupo 3). Procedeu-se à mensuração do pH das amostras após diluição a 1%. Além da estatística descritiva, foram usados os testes de qui-quadrado Persons/Yates e t de Student, com nível de significância mínimo de 5%. Para a estatística inferencial, foram usados os testes de Wilcoxon-Mann-Whitney, exato de Fisher e Kruskal-Wallis. Resultados: O pH variou consideravelmente entre as formas líquida e em barra, com pHs menores (de 4,4 a 7,9) nos líquidos (p < 0,05). Os sindets mostraram pHs próximos ao ideal (levemente ácidos) e os antibacterianos apresentaram os maiores pHs (até 11,34) (p < 0.05). Apenas dois dos sabonetes analisados apresentavam no rótulo a indicação do pH. Conclusões: A observação do pH dos sabonetes infantis pelos médicos e usuários é importante, haja vista a grande variabilidade de valores de pH encontrados. Além disso, os sabonetes líquidos, e especialmente os sindets, são os mais recomendados para uso em recém-nascidos e lactentes com pele sensível, de forma a garantir a eficácia da barreira cutânea.

Abstract Objective: To evaluate the pH value of children's antibacterial soaps and syndets used in children's baths and verify whether there is information regarding pH on the product label. Methods: Quantitative, cross-sectional, analytical observational study that included ninety soap samples, both in bar and liquid presentations, as follows: 67 children's soap (group 1), 17 antibacterial soaps (group 2), and 6 syndets (group 3). Each sample had its pH measured after 1% dilution. In addition to descriptive statistics, the Pearson-Yates chi-squared test and Student's t-tests were applied, considering the minimal significance level of 5%. The Wilcoxon-Mann-Whitney test, Fisher's exact test, and the Kruskal-Wallis test were used for inferential statistics. Results: The pH levels varied considerably between liquid and bar presentations, with lower levels (4.4-7.9) found for the liquids (p < 0.05). Syndets showed pH levels close to the ideal (slightly acid) and the antibacterial soaps showed the highest pH levels (up to 11.34) (p < 0.05). Only two of the soaps included in the study had information about their pH levels on the product packaging. Conclusions: Knowledge of the pH of children's soap by doctors and users is important, considering the great pH variability found in this study. Moreover, liquid soaps, and especially syndets, are the most recommended for the sensitive skin of neonates and infants, in order to guarantee skin barrier efficacy.

A paraqueratose granular é alteração da queratinização, primeiramente descrita em adultos, caracterizada por pápulas e placas hiperqueratósicas nas áreas intertriginosas. Os autores descrevem seis casos de paraqueratose granular em crianças. Um paciente apresentava lesões nas regiões glúteas, dois em ambas as axilas e região cervical (apresentações inéditas na literatura). Três pacientes apresentavam lesões em pregas inguinais. Realizam também revisão da literatura e discutem a possível etiologia dessa rara dermatose.

Granular parakeratosis is an alteration of keratinization that was first described in adults. It is characterized by hiperkeratotic plaques and papules in intertriginous areas. The authors describe six cases of granular parakeratosis in children. One patient had lesions on the buttocks; two children presented papules in both axillae and cervical region (presentations never described before in the literature). The remaining three patients presented with lesions in the inguinal folds. Review of the literature and discussion on the pathogenesis of this rare dermatosis are presented.

FUNDAMENTOS: Desde o início da epidemia da Aids as dermatoses têm sido freqüentemente descritas em pacientes com essa doença, com relatos de casos atípicos e estudos de séries de pacientes adultos; no entanto, há poucas publicações sobre alterações cutâneas em pacientes pediátricos com Aids. OBJETIVOS: Estudo prospectivo para avaliar a presença de dermatoses em 40 pacientes pediátricos com Aids. MÉTODOS: Quarenta pacientes, com idade inferior a 13 anos e portadores de Aids, foram estudados por um período de seis meses para avaliação de: número de alterações dermatológicas; suas características clínicas; distribuição conforme as categorias clínico-imunológicas e o valor da carga viral. RESULTADOS: A prevalência de dermatoses foi de 82,4%, na primeira consulta, e, no acompanhamento longitudinal, 92,5% dos pacientes tiveram alterações dermatológicas, com proporção de cinco diagnósticos por doente. As crianças com classificação clínico-imunológica grave e carga viral acima de 100.000 cópias/ml apresentaram maior número de alterações dermatológicas quando comparadas àquelas das categorias clínico-imunológicas leves. A proporção de diagnósticos por paciente na categoria clínica C foi de 6,8 e na A de 3,6; na categoria imunológica grave, de sete, e na leve de 3,7; e na carga viral > 100.000 de 7,3, e na < 100.000 de 4,2 (todos com significância estatística). CONCLUSÃO: As dermatoses foram freqüentes nas crianças com Aids e ocorreram em maior número nos pacientes pertencentes às categorias graves. A elevada freqüência de alterações da pele nos pacientes pediátricos com a doença indica ser imprescindível sua avaliação dermatológica minuciosa e freqüente.

BACKGROUND: Ever since the beginning of the Acquired Immunodeficiency Syndrome (Aids) epidemic, a number of dermatoses have been frequently described in patients with this disease. In the literature, case reports and studies in series of adult patients with atypical lesions are frequent found. By contrast, there are few reports on cutaneous alterations in pediatric patients with Aids. OBJECTIVE: The purpose of the following prospective survey is to evaluate the presence of dermatoses in 40 pediatric patients with Aids. METHOD: Forty patients diagnosed with Aids and less than 13 years of age were seen for six months. The following features were evaluated: number of dermatoses, clinical characteristics, distribution in accordance with the clinical-immunological categories in relation to CD4 T-lymphocyte and viral load values. RESULTS: The prevalence of dermatoses in the first evaluation was 82.4%. In a longitudinal follow-up, 92.5% of the patients developed some kind of skin problem. Children belonging to the worst clinical-immunological category, with a viral load higher than 100,000 copies/ml showed a higher number of dermatoses when compared to the mildest categories. The number of dermatologic alterations per patient was 6.8 in clinical category C and 3.6 in A. In the immunological severe category, it was 7.0, while in the mild one, it was 3.7. For viral load > 100,000, the number was 7.3, and for < 100,000, it was 4,2 (all with statistical significance). CONCLUSION: Dermatological alterations were frequent and directly related to advanced stages of Aids in pediatric patients. Higher frequencies of skin alterations in pediatric patients with Aids indicate that it is necessary to perform careful and frequent dermatological examinations of these patients.

Patients with AIDS have a high incidence of skin problems due to the immunosuppression and malnourishment that are inherent to the progression of this disease. Clinical manifestation of these skin lesions and their severity are different in AIDS patients. We made a prospective study of five cases of measles in children with HIV infection during a community outbreak, and there were typical as well as atypical forms of the disease, including one case with negative serology. There were pulmonary complications, but none of the patients died. The anti-retroviral treatment may have contributed to the decrease in measles morbidity in these pediatric AIDS patients.

Seis casos de Tinea nigra diagnosticados no Serviço de Dermatopediatria do Hospital de Clínicas de Curitiba, UFPR, no período entre 1978 e 2001 são descritos. Trata-se de quatro crianças em idade escolar que tiveram contato com areia da praia e dois adolescentes. Em um dos casos as lesões são de localização plantar bilateral, configurando o primeiro desse tipo relatado na literatura nacional.

Six cases of Tinea nigra attended at our Pediatric Dermatology Clinic in Hospital de Clínicas de Curitiba - UFPR from 1978 to 2001 are presented. Four cases are schoolchildren and had contact with sand on a beach and two are adolescents. One with bilateral plantar lesions represents the first case reported in national literature.