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Abstract Background Epidermolysis bullosa (EB) is characterized by skin fragility and blistering. In Brazil, the diagnosis is usually obtained through immunomapping, which involves a skin biopsy. Most recently, whole exome sequencing (WES) has become an important tool for the diagnosis of the subtypes of EB, providing information on prognosis as well as allowing appropriate genetic counseling for the families. Objective To compare the results of immunomapping and molecular analysis and to describe the characteristics of a Brazilian cohort of patients with EB. Methods Patients were submitted to clinical evaluation and WES using peripheral blood samples. WES results were compared to those obtained from immunomapping testing from skin biopsies. Results 67 patients from 60 families were classified: 47 patients with recessive dystrophic EB (DEB), 4 with dominant DEB, 15 with EB simplex (EBS), and 1 with junctional EB (JEB). Novel causative variants were: 10/60 (16%) in COL7A1 associated with recessive DEB and 3 other variants in dominant DEB; one homozygous variant in KRT5 and another homozygous variant in PLEC, both associated with EBS. Immunomapping was available for 59 of the 67 patients and the results were concordant with exome results in 37 (62%), discordant in 13 (22%), and inconclusive in 9 patients (15%). Study limitations Even though EB is a rare disease, for statistical purposes, the number of patients evaluated by this cohort can still be considered limited; other than that, there was a significant difference between the proportion of types of EB (only one case with JEB, against more than 50 with DEB), which unfortunately represents a selection bias. Also, for a small subset of families, segregation (usually through Sanger sequencing) was not an option, usually due to deceased or unknown parent status (mostly the father). Conclusion Although immunomapping has been useful in services where molecular studies are not available, this invasive method may provide a misdiagnosis or an inconclusive result in about 1/3 of the patients. This study shows that WES is an effective method for the diagnosis and genetic counseling of EB patients. (EB blistering Brazil biopsy recently (WES samples biopsies 6 classified , (DEB) EBS, EBS (EBS) JEB. JEB . (JEB) 1060 10 10/6 16% 16 (16% COLA COL A COL7A KRT PLEC 5 62%, 62 62% (62%) 22%, 22 22% (22%) 15%. 15% (15%) disease purposes limited only DEB) bias Also option mostly father. father father) 1/ (DEB (EBS (JEB 106 10/ (16 (62% 2 (22% (15% (1 (62 (22 (15 ( (6 (2

https://doi.org/10.1016/j.abd.2023.07.002

Hereditary epidermolysis bullosa: clinical-epidemiological profile of 278 patients at a tertiary hospital in São Paulo, Brazil bullosa clinicalepidemiological clinical epidemiological 27 Paulo 2
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Thien, Chan I.

; Bessa, Vanessa Rolim

; Miotto, Isadora Zago

; Samorano, Luciana Paula

; Rivitti-Machado, Maria Cecília

; Oliveira, Zilda Najjar Prado de

Anais Brasileiros de Dermatologia

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2024, Volume 99 N. 3 Pages 380 - 390

Abstract: En | Text: En | PDF: En

Abstract Background Epidermolysis bullosa (EB) is a group of rare hereditary diseases, characterized by fragility of the skin and mucous membranes. Epidemiological data on EB in Brazil are scarce. Objectives To describe epidemiological aspects of patients with EB diagnosed in the Dermatology Department of a tertiary hospital, from 2000 to 2022. Methods An observational and retrospective study was conducted through the analysis of medical records. The evaluated data included clinical form, sex, family history, consanguinity, age at diagnosis, current age, time of follow-up, comorbidities, histopathology and immunomapping, presence of EB nevi and squamous cell carcinomas (SCC), cause of and age at death. Results Of 309 patients with hereditary EB, 278 were included. The most common type was dystrophic EB (DEB), with 73% (28.4% dominant DEB, 31.7% recessive DEB and 12.9% pruriginous DEB). Other types were junctional EB with 9.4%, EB simplex with 16.5% and Kindler EB with 1.1%. Women accounted for 53% and men for 47% of cases. Family history was found in 35% and consanguinity in 11%. The mean age at diagnosis was 10.8 years and the current age was 26 years. The mean time of follow-up was nine years. Esophageal stenosis affected 14%, dental alterations affected 36%, malnutrition 13% and anemia 29%. During diagnostic investigation, 72.6% underwent histopathological examination and 92% underwent immunomapping. EB nevi were identified in 17%. Nine patients had SCC. Eleven patients died. Study limitations Insufficient data included to medical records, loss to follow-up, and unavailability of genetic testing. Conclusions In this study, dystrophic EB predominated and the need for multidisciplinary care for comorbidities and complications was highlighted. (EB diseases membranes scarce hospital 200 2022 records form sex followup, followup follow up, up immunomapping SCC, SCC , (SCC) death 30 27 (DEB) 73 28.4% 284 28 4 (28.4 317 31 7 31.7 129 12 9 12.9 DEB. . DEB) 94 9.4% 165 16 5 16.5 11 1 1.1% 53 47 cases 35 11% 108 10 8 10. 2 14 14% 36 36% 13 29 29% investigation 726 72 6 72.6 92 17 17% died testing highlighted 20 202 (SCC 3 (DEB 28.4 (28. 31. 12. 9.4 16. 1.1 72. 28. (28 9. 1. (2 (

https://doi.org/10.1016/j.abd.2023.06.009

Consensus on the therapeutic management of atopic dermatitis ‒ Brazilian Society of Dermatology: an update on phototherapy and systemic therapy using e-Delphi technique Dermatology eDelphi e Delphi
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Orfali, Raquel Leao

; Lorenzini, Daniel

; Bressan, Aline

; Tanaka, Anber Ancel

; Cerqueira, Ana Maria Mósca de

; Hirayama, André da Silva

; Ramos, Andréa Machado Coelho

; Proença, Carolina Contin

; Silva, Claudia Marcia de Resende

; Laczynski, Cristina Marta Maria

; Carneiro, Francisca Regina

; Duarte, Gleison

; Hans Filho, Gunter

; Gonçalves, Heitor de Sá

; Melo, Ligia Pessoa de

; Azulay-Abulafia, Luna

; Weber, Magda Blessmann

; Rivitti-Machado, Maria Cecília

; Zaniboni, Mariana Colombini

; Ogawa, Marília

; Pires, Mario Cezar

; Ianhez, Mayra

; Felix, Paulo Antonio Oldani

; Bonamigo, Renan

; Takaoka, Roberto

; Lazzarini, Rosana

; Cestari, Silmara

; Mayor, Silvia Assumpção Soutto

; Cestari, Tania

; Oliveira, Zilda Najjar Prado de

; Spuls, Phyllis I.

; Gerbens, Louise A.A.

; Aoki, Valeria

Anais Brasileiros de Dermatologia

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2023, Volume 98 N. 6 Pages 814 - 836

Abstract: En | Text: En | PDF: En

Abstract This publication is an update of the “Consensus on the therapeutic management of atopic dermatitis - Brazilian Society of Dermatology” published in 2019, considering the novel, targeted-oriented systemic therapies for atopic dermatitis. The initial recommendations of the current consensus for systemic treatment of patients with atopic dermatitis were based on a recent review of scientific published data and a consensus was reached after voting. The Brazilian Society of Dermatology invited 31 experts from all regions of Brazil and 2 international experts on atopic dermatitis who fully contributed to the process. The methods included an e-Delphi study to avoid bias, a literature search and a final consensus meeting. The authors added novel approved drugs in Brazil and the indication for phototherapy and systemic therapy for AD. The therapeutical response to systemic treatment is hereby reported in a suitable form for clinical practice and is also part of this updated manuscript. Consensus 2019 targetedoriented targeted oriented voting 3 process eDelphi e Delphi bias meeting AD manuscript 201 20

https://doi.org/10.1016/j.abd.2023.04.003

Pediatric dermatoses pattern at a Brazilian reference center
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Miotto, Isadora Zago

; Bessa, Vanessa Rolim

; Vasconcelos, Luana Barreto de Almeida

; Samorano, Luciana Paula

; Rivitti-Machado, Maria Cecília

; Oliveira, Zilda Najjar Prado de

Jornal de Pediatria

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apr 2021, Volume 97 N. 2 Pages 211 - 218

Abstract: En | Text: En | PDF: En

Abstract Objective: The aim of this study was to identify the pattern of pediatric dermatoses of patients evaluated at a dermatologic clinic of a reference center in Brazil and to compare these results to similar surveys conducted in other countries. Methods: A retrospective study was performed of patients up to 18 years old, evaluated at a dermatologic clinic between January 1, 2017 and December 31, 2017. Variables collected for analysis included age, gender, dermatological diagnosis, multidisciplinary follow-up, hospitalization, and complementary exams. Results: A total of 2330 patients were included for analysis, with a mean age of 9.7 years. 295 patients were diagnosed with more than one skin disease, leading to a total of 2668 diagnoses. Skin diseases were organized into categories and inflammatory dermatoses corresponded to the largest group (31.2%), mostly due to atopic dermatitis (18.3%). The other main categories were: genodermatoses (14.2%), infectious diseases (12.6%), adnexal disorders (12.5%), cysts and neoplasms (10.7%), and vascular disorders (7.0%). Fifty-six patients needed to be admitted to the dermatology ward; 25 of them (44.6%) for management of worsening of the skin disease, mainly atopic dermatitis, psoriasis, and drug reactions. There were 885 biopsies performed in 38.0% of the subjects and 751 patients (32.2%) required multidisciplinary care; most of them had some genodermatoses. Conclusions: Dermatologic disorders are very common in the pediatric age group and differ from those in adults, suffering influence from cultural, ethnic, socioeconomic, and environmental factors. Knowing the magnitude and distribution of these dermatoses is important to better plan healthcare policies.

https://doi.org/10.1016/j.jped.2020.02.002 150 downloads

CHILD syndrome: successful treatment of skin lesions with topical lovastatin and cholesterol lotion
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Sandoval, Karina Romero

; Machado, Maria Cecília Rivitti

; Oliveira, Zilda Najjar Prado de

; Nico, Marcello Menta Simonsen

Anais Brasileiros de Dermatologia

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may 2019, Volume 94 N. 3 Pages 341 - 343

Abstract: En | Text: En | PDF: En

Abstract: CHILD syndrome (Congenital Hemidysplasia, Ichthyosiform erythroderma, Limb Defects) is a rare X-linked dominant disease. The authors report a 2-month-old patient presenting with typical features of CHILD syndrome that was treated with a topical solution containing cholesterol and lovastatin, with complete clearance of her CHILD nevus. The changes in skin lipid metabolism that explain the CHILD ichthyosiform nevus and their correction through topical application of cholesterol and lovastatin are discussed.

https://doi.org/10.1590/abd1806-4841.20198789 1165 downloads

Consensus on the therapeutic management of atopic dermatitis - Brazilian Society of Dermatology
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Aoki, Valeria

; Lorenzini, Daniel

; Orfali, Raquel Leão

; Zaniboni, Mariana Colombini

; Oliveira, Zilda Najjar Prado de

; Rivitti-Machado, Maria Cecília

; Takaoka, Roberto

; Weber, Magda Blessmann

; Cestari, Tania

; Gontijo, Bernardo

; Ramos, Andrea Machado Coelho

; Silva, Claudia Marcia de Resende

; Cestari, Silmara da Costa Pereira

; Souto-Mayor, Silvia

; Carneiro, Francisca Regina

; Cerqueira, Ana Maria Mosca de

; Laczynski, Cristina

; Pires, Mario Cezar

Anais Brasileiros de Dermatologia

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apr 2019, Volume 94 N. 2 Pages 67 - 75

Abstract: En | Text: En | PDF: En

Abstract: BACKGROUND: Atopic dermatitis is a highly prevalent inflammatory and pruritic dermatosis with a multifactorial etiology, which includes skin barrier defects, immune dysfunction, and microbiome alterations. Atopic dermatitis is mediated by genetic, environmental, and psychological factors and requires therapeutic management that covers all the aspects of its complex pathogenesis. OBJECTIVES: The aim of this article is to present the experience, opinions, and recommendations of Brazilian dermatology experts regarding the therapeutic management of atopic dermatitis. METHODS: Eighteen experts from 10 university hospitals with experience in atopic dermatitis were appointed by the Brazilian Society of Dermatology to organize a consensus on the therapeutic management of atopic dermatitis. The 18 experts answered an online questionnaire with 14 questions related to the treatment of atopic dermatitis. Afterwards, they analyzed the recent international guidelines on atopic dermatitis of the American Academy of Dermatology, published in 2014, and of the European Academy of Dermatology and Venereology, published in 2018. Consensus was defined as approval by at least 70% of the panel. RESULTS/CONCLUSION: The experts stated that the therapeutic management of atopic dermatitis is based on skin hydration, topical anti-inflammatory agents, avoidance of triggering factors, and educational programs. Systemic therapy, based on immunosuppressive agents, is only indicated for severe refractory disease and after failure of topical therapy. Early detection and treatment of secondary bacterial and viral infections is mandatory, and hospitalization may be needed to control atopic dermatitis flares. Novel target-oriented drugs such as immunobiologicals are invaluable therapeutic agents for atopic dermatitis.

https://doi.org/10.1590/abd1806-4841.2019940210 4086 downloads

Consensus on the diagnostic and therapeutic management of chronic spontaneous urticaria in adults - Brazilian Society of Dermatology
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Criado, Paulo Ricardo

; Maruta, Celina Wakisaka

; Alchorne, Alice de Oliveira de Avelar

; Ramos, Andréa Machado Coelho

; Gontijo, Bernardo

; Santos, Josemir Belo dos

; Martins, Luis Eduardo Agner Machado

; Rivitti-Machado, Maria Cecília

; Silvares, Maria Regina Cavariani

; Pires, Mario Cezar

; Souza, Patricia Karla de

; Orfali, Raquel Leão

; Bonamigo, Renan Rangel

; Bedrikow, Roberta Buense

; Criado, Roberta Fachini Jardim

; Oliveira, Zilda Najjar Prado de

Anais Brasileiros de Dermatologia

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apr 2019, Volume 94 N. 2 Pages 56 - 66

Abstract: En | Text: En | PDF: En

Abstract: Background: Urticarias are frequent diseases, with 15% to 20% of the population presenting at least one acute episode in their lifetime. Urticaria are classified in acute ( ≤ 6 weeks) or chronic (> 6 weeks). They may be induced or spontaneous. Objectives: To verify the diagnostic and therapeutic recommendations in chronic spontaneous urticaria (CSU), according to the experience of Brazilian experts, regarding the available guidelines (international and US). Methods: A questionnaire was sent to Brazilian experts, with questions concerning diagnostic and therapeutic recommendations for CSU in adults. Results: Sixteen Brazilian experts answered the questionnaire related to diagnosis and therapy of CSU in adults and data were analyzed. Final text was written, considering the available guidelines (International and US), adapted to the medical practices in Brazil. Diagnostic work up in CSU is rarely necessary. Biopsy of skin lesion and histopathology may be indicated to rule out other diseases, such as, urticarial vasculitis. Other laboratory tests, such as complete blood count, CRP, ESR and thyroid screening. Treatment of CSU includes second-generation anti-histamines (sgAH) at licensed doses, sgAH two, three to fourfold doses (non-licensed) and omalizumab. Other drugs, such as, cyclosporine, immunomodulatory drugs and immunosuppressants may be indicated (non-licensed and with limited scientific evidence). Conclusions: Most of the Brazilian experts in this study partially agreed with the diagnostic and therapeutic recommendations of the International and US guidelines. They agreed with the use of sgAH at licensed doses. Increase in the dose to fourfold of sgAH may be suggested with restrictions, due to its non-licensed dose. Sedating anti-histamines, as suggested by the US guideline, are indicated by some of the Brazilian experts, due to its availability. Adaptations are mandatory in the treatment of CSU, due to scarce or lack of other therapeutic resources in the public health system in Brazil, such as omalizumab or cyclosporine.

https://doi.org/10.1590/abd1806-4841.2019940209 3823 downloads

Tuberous sclerosis complex: review based on new diagnostic criteria
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Portocarrero, Larissa Karine Leite

; Quental, Klícia Novais

; Samorano, Luciana Paula

; Oliveira, Zilda Najjar Prado de

; Rivitti-Machado, Maria Cecília da Matta

Anais Brasileiros de Dermatologia

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jun 2018, Volume 93 N. 3 Pages 323 - 331

Abstract: En | Text: En | PDF: En

Abstract: Tuberous sclerosis complex is a multisystemic, autosomal dominant genetic disorder with complete penetrance, that can evolve with hamartomas in multiple organs, such as skin, central nervous system, kidney and lung. Due to the wide phenotypic variability, the disease is often not recognized. Tuberous sclerosis complex affects one in 10,000 newborns and most patients are diagnosed during the first 15 months of life. The diagnostic criteria for tuberous sclerosis were reviewed in 2012, at the second International Tuberous Sclerosis Complex Consensus Conference. The diagnosis is based on genetic criteria, by the identification of inactivating pathogenic mutation of tumor suppressor genes TSC1 and TSC2, and clinical criteria, including cutaneous, renal, pulmonary, cardiac and neurological manifestations. The treatment of tuberous sclerosis complex consists, mainly, in management of the symptoms caused by hamartomas and in prevention of organ failure. Multidisciplinary approach is recommended, in order to obtain better clinical outcomes.

https://doi.org/10.1590/abd1806-4841.20186972 5473 downloads

10.

Propranolol for treatment of infantile hemangiomas
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Melo, Juliana Nakano de

; Rotter, Anita

; Rivitti-Machado, Maria Cecília

; Oliveira, Zilda Najjar Prado de

Anais Brasileiros de Dermatologia

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dec 2013, Volume 88 N. 6 Pages 220 - 223

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

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.

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.

https://doi.org/10.1590/abd1806-4841.20131699 8817 downloads

11.

Fotoproteção tópica na infância e na adolescência
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Criado, Paulo Ricardo

; Melo, Juliana Nakano de

; Oliveira, Zilda Najjar Prado de

Jornal de Pediatria

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jun 2012, Volume 88 N. 3 Pages 203 - 210

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

OBJETIVO: A exposição à luz solar na infância ocorre, frequentemente, de forma mais intensa do que em muitos adultos. Dados da literatura comprovam de maneira inequívoca a associação desse comportamento social com o risco de desenvolvimento do melanoma maligno e do câncer cutâneo não melanoma mesmo na vida adulta. Além disso, o fotoenvelhecimento cutâneo é semeado já na infância com a exposição solar inadequada. Esta revisão tem como objetivo orientar os pediatras nas medidas adequadas de fotoproteção tópica nas crianças e adolescentes, o que irá alterar de maneira positiva o futuro desses pacientes. FONTES DOS DADOS: Realizou-se uma revisão da literatura indexada na base de dados MEDLINE/PubMed entre os anos de 1999 e 2012 sobre fotoproteção na infância, selecionando-se como fonte os artigos de revisão mais relevantes, do ponto de vista de abrangência do tema fotoproteção em crianças e adolescentes, fotoproteção e vitamina D, fototerapia na neonatologia e impacto no câncer cutâneo, bronzeamento artificial e câncer cutâneo. SÍNTESE DOS DADOS: Crianças e adolescentes devem adotar medidas adequadas de fotoproteção para diminuir o risco de câncer cutâneo melanoma e não melanoma. CONCLUSÕES: Há dados na literatura que suportam a associação de hábitos de exposição solar segura e uso de fotoprotetores tópicos em crianças e adolescentes com a redução da ocorrência do câncer cutâneo.

OBJECTIVE: Exposure to sunlight in childhood is often more intense than in adults. Literature data unequivocally show the association between this social behavior and the risk for developing malignant melanoma and non-melanoma skin cancer, even in adulthood. Furthermore, skin photoaging begins already in childhood through inadequate sun exposure. This review aims to guide pediatricians on appropriate measures of topical photoprotection in children and adolescents, which will positively change the future of these patients. SOURCES: A review of the literature indexed in MEDLINE/PubMed between the years 1999 and 2012 on photoprotection in childhood was conducted. The most relevant review articles on photoprotection in children and adolescents, photoprotection and vitamin D in neonatal phototherapy and impact on skin cancer, artificial tanning and skin cancer were selected as sources. SUMMARY OF THE FINDINGS: Children and adolescents should adopt appropriate measures of photoprotection in order to decrease the risk of melanoma and non-melanoma skin cancer. CONCLUSIONS: There are published data that support the association between sun exposure habits and safe use of topical sunscreens in children and adolescents on the one hand and a reduced occurrence of skin cancer on the other.

https://doi.org/10.2223/JPED.2194 14797 downloads Cited 2 times in SciELO

12.

Nevo da epidermólise bolhosa: aspectos clínicos, dermatoscópicos e histológicos em um caso de portador da forma distrófica recessiva
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Melo, Juliana Nakano de

; Teruya, Priscila Yoshie

; Machado, Maria Cecília Rivitti

; Valente, Neusa Sakai

; Sotto, Mirian Nacagami

; Oliveira, Zilda Najjar Prado de

Anais Brasileiros de Dermatologia

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aug 2011, Volume 86 N. 4 Pages 743 - 746

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

As lesões melanocíticas adquiridas podem apresentar aspecto clínico não-usual em pacientes portadores de epidermólise bolhosa hereditária. Essas lesões são conhecidas como "nevos EB" e, muitas vezes, constituem um desafio diagnóstico ao dermatologista por apresentarem características clínicas, dermatoscópicas e histopatológicas semelhantes às encontradas no melanoma. Não são exclusivas de nenhuma forma de epidermólise bolhosa e têm sua frequência aumentada na infância. Relata-se o caso de um doente do sexo masculino, de 6 meses de idade, portador da forma distrófica recessiva da doença, com lesão pigmentada de rápido crescimento na coxa esquerda. Optou-se por seguimento clínico da lesão, considerando que os aspectos clínicos, dermatoscópicos e histológicos eram compatíveis com a descrição de outros casos de nevo EB previamente descritos

Acquired melanocytic lesions may present unusual clinical features in all forms of hereditary epidermolysis bullosa. These lesions are known as "EB nevi", and often pose a diagnostic challenge for dermatologists given their resemblance - clinically, dermoscopically and histologically - to melanoma. The lesions have been reported in all types of hereditary EB, most of them in childhood. We report the case of a 6-month-old boy suffering from recessive dystrophic epidermolysis bullosa (RDEB) that presented as a large pigmented lesion on his left thigh. We decided to monitor the lesion closely since we considered that the clinical and pathological aspects of the lesion were compatible with the description of other previously reported cases of EB nevi

7780 downloads

13.

Prevenção e cuidados com a pele da criança e do recém-nascido
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Fernandes, Juliana Dumêt

; Machado, Maria Cecília Rivitti

; Oliveira, Zilda Najjar Prado de

Anais Brasileiros de Dermatologia

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feb 2011, Volume 86 N. 1 Pages 102 - 110

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

A pele do neonato é submetida a um progressivo processo de adaptação ao ambiente extrauterino, para o qual cuidados especiais se tornam necessários. A sua pele caracteriza-se por ser sensível, fina e frágil. A imaturidade da sua barreira epidérmica diminui significativamente a defesa contra a excessiva proliferação microbiana, torna a pele mais susceptível ao trauma e à toxicidade por absorção percutânea de drogas. Devido às características próprias da pele do recém-nascido (RN), de lactentes e de crianças, o uso dos produtos cosméticos destinados à sua higiene e proteção requer um cuidado especial. Com o intuito de preservar a integridade da pele neonatal e infantil, este artigo revisou os cuidados preventivos básicos que se devem ter com a pele dos bebês quanto à higiene, ao banho, ao uso de agentes de limpeza, a produtos tópicos e a sua toxicidade percutânea.

Neonatal skin suffers a progressive adaptation to the extrauterine environment and special care is needed during this period. This skin is very sensitive, thin and fragile. Immaturity of the epidermal barrier reduces the defense against the excessive proliferation of microbes and makes the skin more vulnerable to trauma and percutaneous drug toxicity. Because of the peculiar characteristics of newborn, infant and children's skin, the use of cosmetic products designed for hygiene and protection requires caution. In order to preserve the integrity of neonatal and child's skin, this article reviewed basic preventive care practices in relation to hygiene, bathing, cleansing agents, topical products and their percutaneous toxicity.

91993 downloads

14.

Imunomapeamento nas epidermólises bolhosas hereditárias
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Oliveira, Zilda Najjar Prado de

; Périgo, Alexandre M.

; Fukumori, Lígia M. I.

; Aoki, Valéria

Anais Brasileiros de Dermatologia

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dec 2010, Volume 85 N. 6 Pages 856 - 861

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

O imunomapeamento, uma técnica de imunofluorescência, é o método atual mais utilizado para o diagnóstico laboratorial e a diferenciação dos principais tipos de epidermólise bolhosa hereditária, uma vez que determina o plano de clivagem na junção dermo-epidérmica das doenças mecano-bolhosas.

Immunological mapping, an immunofluorescence technique, is currently the method most used to diagnose and differentiate the principal types of hereditary epidermolysis bullosa, since this technique is capable of determining the level of cleavage of this mechanobullous disease.

20655 downloads

15.

Estudo da imunofluorescência direta, imunomapeamento e microscopia ótica na porfiria cutânea tardia
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Vieira, Fátima Mendonça Jorge

; Aoki, Valéria

; Oliveira, Zilda Najjar Prado de

; Martins, José Eduardo Costa

Anais Brasileiros de Dermatologia

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dec 2010, Volume 85 N. 6 Pages 827 - 837

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

FUNDAMENTO: Apesar de a porfiria cutânea tardia ser a mais frequente das porfirias, há poucos estudos que abordam sua fisiopatologia cutânea. OBJETIVO: Avaliar as alterações cutâneas na porfiria cutânea tardia utilizando a microscopia ótica e a imunofluorescência direta, antes e depois do tratamento com cloroquina. Realizar o imunomapeamento antigênico da bolha para estudo do seu nível de clivagem. MÉTODOS: Relata-se a microscopia ótica e imunofluorescência direta de 28 pacientes em três fases diferentes: 23 pacientes com porfiria ativa antes do tratamento (Fase A), sete pacientes com remissão clínica durante o tratamento (Fase B) e oito pacientes com remissão bioquímica (Fase C). O imunomapeamento foi realizado em sete pacientes. RESULTADOS: Na porfiria ativa, a imunofluorescência direta demonstrou fluorescência homogênea e intensa no interior e na parede dos vasos e na junção dermoepidérmica. Na remissão clínica (Fase B) e na remissão bioquímica (Fase C), o depósito de imunoglobulinas se manteve, mas o depósito de complemento apresentou diminuição na maioria. O imunomapeamento não demonstrou plano de clivagem fixo. CONCLUSÃO: Não houve correlação entre a resposta clínica e os depósitos de imunoglobulinas. A diminuição do complemento favorece a hipótese de que a ativação da cascata do complemento representa uma via adicional que leva à lesão endotelial.

BACKGROUND: Even though porphyria cutanea tarda is the most frequent type of porphyria, there are few studies about its cutaneous physiopathology. OBJECTIVE: To evaluate skin changes in porphyria cutanea tarda using light microscopy and direct immunofluorescence before and after treatment with chloroquine. To perform antigen immunomapping of bullae to study their level of cleavage. METHODS: Light microscopy and direct immunofluorescence of 28 patients are reported in three different phases: 23 patients with active porphyria before treatment (Phase A), 7 patients with clinical remission during treatment (Phase B), and 8 patients with biochemical remission (Phase C). Immunomapping was performed on 7 patients. RESULTS: In active porphyria, direct immunofluorescence showed homogenous and intense fluorescence on the inside and on the walls of blood vessels as well as in the dermal-epidermal junction. In clinical remission (Phase B) and biochemical remission (Phase C), the deposit of immunoglobulins was maintained, but the deposit of complement was reduced in most cases. Immunomapping revealed no standard cleavage plane. CONCLUSION: No correlation was observed between clinical response and immunoglobulin deposits. The reduction of complement favors the hypothesis that activation of the complement cascade represents an additional pathway that leads to endothelial damage.

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