Ultrastructural aspects of mucosas in endemic pemphigus foliaceus.

OBJECTIVE: To investigate whether ultrastructural changes present in clinically normal oral mucosa could occur in the mucosas of patients with endemic pemphigus foliaceus (EPF) or fogo selvagem (wildfire). PATIENTS: Surgical biopsy specimens were taken from the foreskin of 8 patients with EPF and 3 control subjects, the uterine cervix and vaginal wall of 9 patients with EPF and 2 controls, and the oral mucosa of 5 patients with EPF and 4 controls. The patients received a clinical and histopathologic diagnosis of EPF and all had clinically normal oral and genital mucosas. RESULTS: In the patients with EPF, widening of the intercellular spaces and distended, elongated cytoplasmic projections, the tips of which contained desmosomes and were sometimes disassembled, were evident in all 4 regions studied. At the periphery of the spinous cells, cytoplasmic vesicles apparently containing intact or fragments of desmosomes or half-desmosomes were seen. CONCLUSIONS: The ultrastructural lesions found in the mucosas studied are similar to those previously described in the literature for the oral mucosa of patients with EPF. In the cases of EPF, even though the desmosomal changes occurred in all epithelial layers, blisters did not occur in the mucosas by possible coexpression of desmoglein 1 and desmoglein 3.

Comparison between anti-PGL-I serology and Mitsuda reaction: clinical reading, microscopic findings and immunohistochemical analysis.

The lepromin test, serum IgM antibodies against Mycobacterium leprae and in situ observations of T cell subsets in biopsies of Mitsuda reaction using monoclonal antibodies were performed on 44 untreated leprosy patients belonging to various classifications of the disease. The Mitsuda reaction was accessed clinically and histologically after 28 days. Clinical reading and histological analysis of Mitsuda reaction showed good agreement. The high positivity in clinical reading correlated with compact granulomas in histology. There is a graduation of Mitsuda reaction that follows the immunological spectrum of the disease. The histological study of Mitsuda reaction is valuable to confirm the immunological condition in doubtful clinical reaction. Anti-PGL-I IgM levels correlated with disease classification, increasing from the tuberculoid towards the lepromatous pole of the disease spectrum. There was an inverse correlation between serum IgM antibody levels and clinical and histological reading of Mitsuda reaction. There were no statistical difference in quantities and distribution of CD4+ and CD8+ T cells in all Mitsuda reactions. The pattern of cellular content of Mitsuda reaction could not be related to the T cells.

Hereditary angioedema type III (estrogen-dependent) report of three cases and literature review.

In this article, three cases of hereditary angioedema (HAE) type III (estrogen-dependent or with normal C1 inhibitor) are reported. The HAE was initially described in women of the same family in association with high-leveled estrogenic conditions such as the use of oral contraceptives and pregnancy. There is no change in the C1 inhibitor as happens in other types of hereditary angioedema, and mutations are observed in the encoding gene of the XII factor of coagulation in several patients. The current diagnosis is mainly clinical and treatment consists in the suspension of the triggering factors and control of acute symptoms. A brief review of physiopathology, clinical features, genetic alterations and treatment are also presented.

Cutaneous and pulmonary paracoccidioidomycosis in a patient with a malignant visceral tumor.

Paracoccidioidomycosis is a systemic mycosis caused by the fungus Paracoccidioides brasiliensis that is characterized by polymorphous clinical manifestations principally affecting the skin, mucous membranes, lungs, lymph nodes, adrenal glands and the central nervous system. Depending on the site of inoculation and the individual's immunological status, the disease may take various different forms, affecting the skin, lymph nodes, viscera or a combination of these. The present report describes a patient with extensive cutaneous and pulmonary paracoccidioidomycosis, with disseminated papules and pustules, fever and pulmonary symptoms, probably related to immunosuppression caused by a renal carcinoma.

Risk factors for nonmelanoma skin cancer in Taubaté, São Paulo, Brazil: a case-control study.

OBJECTIVE: To identify factors associated with non-melanoma skin cancer in the city of Taubaté, São Paulo, Brazil. METHODS: Hospital-based case-control study with individuals residing in Taubaté, treated between January 2005 and December 2006. The subjects were matched 1:1 according to gender and age. Age, gender, phototype, European descent, time of residence, sun exposure, time (in years), number of hours and time of occupational and non-occupational sun exposure, photoprotection, family history and photodamage were independent variables. A hierarchical logistic regression was used at three levels. The model adjustment was performed using the Hosmer-Lemeshow test and its accuracy was verified by ROC curve. The significance level was p < 0.05. RESULTS: There were 132 cases and 132 controls. Phototype with OR = 3.14 (95% CI 1.79-5.49), hours of occupational 1.76 (1.04-2.99) and non-occupational sun exposure 1.80 (0.98-3.29) and family history 2.10 (1.13-3.93) were the variables in the final model. Hosmer-Lemeshow test, p = 0.97. Accuracy 70% (95% CI 63-76). CONCLUSION: We concluded that fair skin, family history and occupational and non-occupational sun exposure were associated with non-melanoma skin cancer in Taubaté-São Paulo.

Quality of life assessment in patients with chronic urticaria.

BACKGROUND: Chronic urticaria affects patients by interfering with their daily activities, damaging their self-esteem and negatively affecting their interpersonal relationships. Healthcare professionals may underestimate the impact of the condition on patients' quality of life. OBJECTIVES: To evaluate quality of life using one generic and one disease-specific health-related quality of life instrument; to compare quality of life between the different clinical types of chronic urticaria and to evaluate whether angioedema further impairs quality of life. METHODS: Sixty-two patients with chronic urticaria, who had had signs or symptoms of the disease within the preceding week, were divided into groups according to whether they had ordinary chronic urticaria, physical chronic urticaria or mixed chronic urticaria. RESULTS: There was a predominance of women in this study (72.6%). The mean age of patients was 39.8 years and angioedema was associated with chronic urticaria in 75.8% of cases. Associated angioedema was more commonly found in patients with ordinary chronic urticaria (p=0.011) and in women (p=0.024). With respect to the different clinical types, 32.3% of the patients had ordinary chronic urticaria, 27.4% had physical chronic urticaria and 40.3% had mixed chronic urticaria. Mean overall score for the disease-specific questionnaire was 10.4. In this questionnaire, the most affected domains were "symptoms and feelings" and "daily activities", while the most affected domains in the SF-36 were "physical role" and "vitality". Quality of life was found to be impaired in women, in patients of up to 30 years of age, in those attending a first consultation, in those with higher education levels, in patients who had had the disease for up to one year and in those with angioedema. CONCLUSION: Chronic urticaria affects quality of life, as measured using a disease-specific questionnaire and a generic questionnaire. There was no statistically significant difference between the clinical types. The presence of angioedema impaired patients' quality of life even further. There was a statistically significant correlation between the scores obtained using the disease-specific questionnaire and those obtained using the generic questionnaire.

[Familial aquagenic urticaria: report of two cases and literature review]. / Urticária aquagênica familiar: relato de dois casos e revisão da literatura.

Aquagenic urticaria is a rare form of physical urticaria, characterized by pruritic wheals that appear following contact with water, independently of its temperature. There are few reports of cases of aquagenic urticaria, and only five include the familial form. We present the first case of familial aquagenic urticaria in Brazil (mother and daughter). Both patients presented wheals following contact with water, especially when showering, regardless of its temperature. The mother reported onset of urticaria four years before and the daughter presented wheals since birth. For diagnostic purposes, they were submitted to a challenge test with water, and both subjects presented wheals, as well as to tests using ice cubes in plastic bag with dermographometer and challenge tests for cholinergic urticaria, with no appearance of lesions, excluding other forms of physical urticaria.

Hereditary angioedema type III (estrogen-dependent) report of three cases and literature review* / Angioedema hereditario tipo III (estrogeno-dependente): relato de tres casos e revisao da literatura

In this article, three cases of hereditary angioedema (HAE) type III (estrogen-dependent or with normal C1 inhibitor) are reported. The HAE was initially described in women of the same family in association with high-leveled estrogenic conditions such as the use of oral contraceptives and pregnancy. There is no change in the C1 inhibitor as happens in other types of hereditary angioedema, and mutations are observed in the encoding gene of the XII factor of coagulation in several patients. The current diagnosis is mainly clinical and treatment consists in the suspension of the triggering factors and control of acute symptoms. A brief review of physiopathology, clinical features, genetic alterations and treatment are also presented.

Neste artigo são relatados três casos de angioedema hereditário do tipo III (estrógeno-dependente ou com inibidor de C1 normal), que foi inicialmente descrito em mulheres da mesma família, em associação com condições de alto nível estrogênico, como uso de anticoncepcionais orais e gravidez. Não há alteração do inibidor de C1, como acontece nos outros tipos de angioedema hereditário, e são observadas mutações no gene codificador do fator XII da coagulação em várias pacientes. O diagnóstico atualmente é eminentemente clínico e o tratamento consiste na suspensão dos fatores desencadeantes e controle dos sintomas agudos. Também é apresentada breve revisão da fisiopatogenia, quadro clínico, alterações genéticas e tratamento.

Paracoccidioidomicose cutânea disseminada e pulmonar em paciente portador de neoplasia maligna visceral / Cutaneous and pulmonary paracoccidioidomycosis in a patient with a malignant visceral tumor

Paracoccidioidomicose é doença causada pelo fungo Paracoccidioides brasiliensis, caracterizada por quadro polimórfico e acometimento preferencial de pele, mucosas, pulmões, linfonodos, adrenais e sistema nervoso. De acordo com o local de inoculação e o estado imunológico do indivíduo, ocorrem as diversas formas da doença: tegumentar, linfonodular, visceral e mista. Relatamos caso de paciente com quadro de paracoccidioidomicose mista (tegumentar e pulmonar), com lesões cutâneas caracterizadas por pápulas e pústulas disseminadas e sintomas sistêmicos, possivelmente associada a imunossupressão causada por neoplasia maligna visceral.

Paracoccidioidomycosis is a systemic mycosis caused by the fungus Paracoccidioides brasiliensis that is characterized by polymorphous clinical manifestations principally affecting the skin, mucous membranes, lungs, lymph nodes, adrenal glands and the central nervous system. Depending on the site of inoculation and the individual's immunological status, the disease may take various different forms, affecting the skin, lymph nodes, viscera or a combination of these. The present report describes a patient with extensive cutaneous and pulmonary paracoccidioidomycosis, with disseminated papules and pustules, fever and pulmonary symptoms, probably related to immunosuppression caused by a renal carcinoma.

Fatores de risco para câncer da pele não melanoma em Taubaté, SP: um estudo caso-controle / Risk factors for nonmelanoma skin cancer in Taubaté, SP: a case-control study

OBJETIVO: Identificar fatores associados ao câncer da pele não melanoma em Taubaté-SP. MÉTODOS: Estudo caso-controle de base hospitalar com residentes em Taubaté atendidos entre 01/01/05 e 31/12/06. Os indivíduos foram combinados 1:1 conforme gênero e idade. Foram variáveis independentes: idade, gênero, fototipo, ascendência europeia, tempo de moradia, exposição solar, tempo (em anos), número de horas e horário de exposição solar ocupacional e não ocupacional, fotoproteção, histórico familiar e fotodano. Foi utilizada a regressão logística hierarquizada em três níveis. O ajuste do modelo foi realizado pelo teste de Hosmer-Lemeshow e sua acurácia foi verificada pela curva ROC. O nível de significância foi p < 0,05. RESULTADOS: Foram 132 casos e 132 controles. Compuseram o modelo final: fototipo com OR = 3,14 (IC 95 por cento 1,79-5,49), horas de exposição solar ocupacional 1,76 (1,04-2,99) e não ocupacional 1,80 (0,98-3,29) e histórico familiar 2,10 (1,13-3,93).Teste de Hosmer-Lemeshow, p = 0,97. Acurácia 70 por cento (IC 95 por cento 63-76). CONCLUSÃO: Pele clara, histórico familiar e exposição solar ocupacional e não ocupacional se associaram ao câncer cutâneo não melanoma em Taubaté-SP.

OBJECTIVE: To identify factors associated with non-melanoma skin cancer in the city of Taubaté, São Paulo, Brazil. METHODS: Hospital-based case-control study with individuals residing in Taubaté, treated between January 2005 and December 2006. The subjects were matched 1:1 according to gender and age. Age, gender, phototype, European descent, time of residence, sun exposure, time (in years), number of hours and time of occupational and non-occupational sun exposure, photoprotection, family history and photodamage were independent variables. A hierarchical logistic regression was used at three levels. The model adjustment was performed using the Hosmer-Lemeshow test and its accuracy was verified by ROC curve. The significance level was p < 0.05. RESULTS: There were 132 cases and 132 controls. Phototype with OR = 3.14 (95 percent CI 1.79-5.49), hours of occupational 1.76 (1.04-2.99) and non-occupational sun exposure 1.80 (0.98-3.29) and family history 2.10 (1.13-3.93) were the variables in the final model. Hosmer-Lemeshow test, p = 0.97. Accuracy 70 percent (95 percent CI 63-76). CONCLUSION: We concluded that fair skin, family history and occupational and non-occupational sun exposure were associated with non-melanoma skin cancer in Taubaté-São Paulo.

Estudo da qualidade de vida nos pacientes com urticária crônica / Quality of life assessment in patients with chronic urticaria

FUNDAMENTOS: A urticária crônica compromete o doente por interferir nas atividades diárias, prejudicar a autoestima e as relações interpessoais. Os profissionais de saúde subestimam seu impacto na qualidade de vida dos doentes. OBJETIVOS: Avaliar a qualidade de vida com questionário específico e genérico. Compará-la entre os tipos clínicos de urticária crônica e avaliar se o angioedema piora a qualidade de vida. MÉTODOS: Participaram 62 doentes com urticária crônica, com sinais e sintomas da doença até 7 dias da consulta, que foram divididos em urticária crônica comum, urticária crônica física e urticária crônica mista. RESULTADOS: Observou-se predominância de mulheres (72,6 por cento), idade média de 39,8 anos, angioedema associado em 75,8 por cento dos doentes. Apresentaram mais angioedema doentes com urticária crônica comum (p=0,011) e mulheres (p=0,024). Quanto aos tipos clínicos, 32,3 por cento apresentaram urticária crônica comum, 27,4 por cento urticária crônica física e 40,3 por cento urticária crônica mista. O escore médio total do questionário específico foi 10,4. No questionário específico, os domínios mais comprometidos foram "Sintomas e sentimentos" e "Atividades diárias", e, no SF-36, "Aspectos físicos" e "Vitalidade". Houve comprometimento da qualidade de vida nas mulheres, nos doentes com até 30 anos, em primeira consulta, nos mais escolarizados, naqueles com até 1 ano de doença e naqueles com angioedema. CONCLUSÃO: A urticária crônica compromete a qualidade de vida medida pelos questionários específico e genérico. Não houve diferença estatisticamente significante na qualidade de vida entre os tipos clínicos. A presença do angioedema conferiu pior qualidade de vida aos doentes. Houve correlação estatisticamente significante entre os escores do questionário específico e do questionário genérico.

BACKGROUND: Chronic urticaria affects patients by interfering with their daily activities, damaging their self-esteem and negatively affecting their interpersonal relationships. Healthcare professionals may underestimate the impact of the condition on patients' quality of life. OBJECTIVES: To evaluate quality of life using one generic and one disease-specific health-related quality of life instrument; to compare quality of life between the different clinical types of chronic urticaria and to evaluate whether angioedema further impairs quality of life. METHODS: Sixty-two patients with chronic urticaria, who had had signs or symptoms of the disease within the preceding week, were divided into groups according to whether they had ordinary chronic urticaria, physical chronic urticaria or mixed chronic urticaria. RESULTS: There was a predominance of women in this study (72.6 percent). The mean age of patients was 39.8 years and angioedema was associated with chronic urticaria in 75.8 percent of cases. Associated angioedema was more commonly found in patients with ordinary chronic urticaria (p=0.011) and in women (p=0.024). With respect to the different clinical types, 32.3 percent of the patients had ordinary chronic urticaria, 27.4 percent had physical chronic urticaria and 40.3 percent had mixed chronic urticaria. Mean overall score for the disease-specific questionnaire was 10.4. In this questionnaire, the most affected domains were "symptoms and feelings" and "daily activities", while the most affected domains in the SF-36 were "physical role" and "vitality". Quality of life was found to be impaired in women, in patients of up to 30 years of age, in those attending a first consultation, in those with higher education levels, in patients who had had the disease for up to one year and in those with angioedema. CONCLUSION: Chronic urticaria affects quality of life, as measured using a disease-specific questionnaire and a generic questionnaire. There was no statistically significant difference between the clinical types. The presence of angioedema impaired patients' quality of life even further. There was a statistically significant correlation between the scores obtained using the disease-specific questionnaire and those obtained using the generic questionnaire.

Urticária aquagênica familiar: relato de dois casos e revisão da literatura / Familial aquagenic urticaria: report of two cases and literature review

Urticária aquagênica é forma rara de urticária física caracterizada por aparecimento de urticas após o contato com água, independente da temperatura. Há poucos casos descritos de urticária aquagênica e, destes, somente cinco da forma familiar. Apresentamos o primeiro relato de urticária aquagênica familiar no Brasil, acometendo mãe e filha. Ambas apresentavam urticas, principalmente após banho de chuveiro, independentemente da temperatura da água. A mãe referia ter o quadro há quatro anos, e a filha, desde o nascimento. Para diagnóstico, foram realizados testes de provocação com água, com aparecimento de lesões em ambas, e testes com dermografômetro, com cubo de gelo envolvido em plástico e de provocação para urticária colinérgica, sem o aparecimento de lesões, excluindo assim outras formas de urticária física.

Aquagenic urticaria is a rare form of physical urticaria, characterized by pruritic wheals that appear following contact with water, independently of its temperature. There are few reports of cases of aquagenic urticaria, and only five include the familial form. We present the first case of familial aquagenic urticaria in Brazil (mother and daughter). Both patients presented wheals following contact with water, especially when showering, regardless of its temperature. The mother reported onset of urticaria four years before and the daughter presented wheals since birth. For diagnostic purposes, they were submitted to a challenge test with water, and both subjects presented wheals, as well as to tests using ice cubes in plastic bag with dermographometer and challenge tests for cholinergic urticaria, with no appearance of lesions, excluding other forms of physical urticaria.

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

A síndrome de Papillon-Lefèvre ou queratodermia transgressiva com periodontopatia é genodermatose rara, com acometimento cutâneo e dentário. As alterações aparecem por volta do primeiro ano de vida, com queratodermia transgressiva palmoplantar associada a periodontites, com perda precoce dos dentes decíduos e permanentes. É freqüentemente associada com infecções bacterianas da pele e de órgãos internos. A histopatologia é inespecífica, sendo o diagnóstico eminentemente clínico.

Papillon-Lefèvre syndrome, or palmoplantar keratoderma with periodontitis, is a rare genodermatosis with cutaneous and dental repercussions. The condition appears at around the first year of life, with transgressive palmar-plantar keratoderma associated with periodontitis, early loss of both deciduous and permanent teeth. It is frequently associated with pyogenic infection of the skin and internal organs. Histopathology is nonspecific, the diagnosis being eminently clinical.

Dapsona como alternativa no tratamento de urticária crônica não responsiva a anti-histamínicos / Dapsone as an alternative to the treatment of chronic urticaria non-responsive to antihistamines

FUNDAMENTOS: A urticária crônica é dermatose que interfere negativamente na qualidade de vida de seus portadores. O tratamento clássico com anti-histamínicos muitas vezes é ineficaz. OBJETIVO: Avaliar a eficácia e a segurança do uso da dapsona no tratamento da urticária crônica não responsiva a anti-histamínicos. METÓDOS: Realizou-se estudo retrospectivo mediante a revisão de prontuários de pacientes atendidos em ambulatório especializado em urticária entre novembro de 1996 e março de 2007. RESULTADOS: Foram avaliados 20 pacientes com urticária crônica de difícil controle, que receberam tratamento com dapsona na dose de 100mg/dia. Associados à dapsona, foram mantidos anti-histamínicos em altas doses, que, isoladamente, não controlavam os sintomas. Quatorze pacientes (70 por cento) responderam com melhora do quadro, observada tanto na diminuição ou desaparecimento das lesões quanto na redução do prurido; três (15 por cento) não obtiveram nenhum sucesso com a medicação; e três (15 por cento) tiveram o tratamento suspenso em decorrência de efeitos colaterais. CONCLUSÃO: Neste estudo, conclui-se que a dapsona é opção segura e eficaz para pacientes com urticária crônica grave não responsiva a anti-histamínicos.

BACKGROUND: Chronic urticaria is a dermatosis that negatively interferes in quality of life of affected individuals. The classic treatment with antihistamines is many times ineffective. OBJECTIVE: To evaluate the efficacy and safety of using dapsone in the treatment of chronic urticaria non-responsive to antihistamines. METHODS: A retrospective study was carried out by reviewing the medical charts of patients seen at an outpatient’s clinic specialized in urticaria, between November 1996 and March 2007. RESULTS: Twenty patients with difficult to control chronic urticaria and who were treated with 100 mg/day of dapsone were evaluated. High doses of antihistamines were maintained and associated with dapsone. Antihistamines alone did not control symptoms. Fourteen patients (70 percent) presented good results, observed in the reduction or disappearance of lesions as well as in reduced pruritus; three patients (15 percent) did not get any success with the medication; and three (15 percent) had the treatment interrupted due to side effects. CONCLUSION: In this study it can be concluded that dapsone is a safe and efficient option for patients presenting severe chronic urticaria non-responsive to antihistamines.

Ginecomastia induzida por anti-histamínicos no tratamento da urticária crônica / Antihistaminic-induced gynecomastia in chronic urticaria treatment

Os anti-histamínicos são drogas muito usadas na prática do dermatologista, sendo a primeira escolha no tratamento da urticária crônica. Os efeitos colaterais mais comuns são os relacionados ao sistema nervoso central. A ginecomastia é decorrente de várias causas, entre elas a indução por drogas. Apresenta-se caso de ginecomastia induzida por anti-histamínico H1,em paciente em tratamento de urticária crônica. A investigação laboratorial e radiológica descartou outras causas para a ginecomastia, que involuiu com a retirada da medicação. Objetiva-se discutir os efeitos colaterais dos anti-histamínicos e apresentar caso de ginecomastia induzida por drogas.

Antihistaminic drugs are very commonly used in dermatological practice, and are the first-line therapy to chronic urticaria. The commonest side effects are related to the central nervous system. Gynecomastia, in turn, may be due to a myriad of disorders, including the use of medication. The case of H1 antihistaminic-induced gynecomastia in a patient undergoing chronic urticaria treatment is reported. Radiological and laboratorial investigations discarded other possible causes for the gynecomastia, which disappeared after removal of the medication. Antihistaminic-related side effects, including gynecomastia, are discussed.

Diagnóstico sorológico da Sífilis / Serological diagnosis of syphilis

Há 80 anos, o assistente do Instituto Vital Brasil Arlindo de Assis publicava extenso artigo denominado ?O immunodiagnostico da syphilis na actualidade? em que dissertava e discutia sobre inúmeras técnicas sorológicas que se desenvolviam no mundo no agora longínquo ano de 1925. Por extenso, não pode ser sabiamente resumido, mas apenas ter citados trechos que revelavam suas preocupações

Anti-phenolic glycolipid-I (PGL-I) determination using blood collection on filter paper in leprosy patients

The authors studied 70 leprosy patients and 20 normal individuals, comparing the traditional sera collection method and the finger prick blood with the conservation on filter paper for specific antibodies against the native phenolic glycolipid-I (PGL-I) from Mycobacterium leprae. The finger prick blood dried on filter paper was eluated in phosphate buffer saline (PBS) containing 0.5 percent gelatin. The classical method for native PGL-I was performed for these eluates, and compared with the antibody determination for sera. It was observed that there is a straight correlation comparing these two methods; although the titles found for the eluates were lower than those obtained for serology. This blood collection method could be useful for investigation of new leprosy cases in field, specially in contacts individuals

Acidentes provocados por Millipede com manifestações dermatológicas: relatos de dois casos / Accidents provoked by Millipede with dermatological manifestations: report of two cases

Mediante a descrição de dois acidentes provocados por artrópodas (Millipede), os autores comentam essa forma de acidente, descrevem o agente envolvido, seu mecanismo de agressão e apresentam referências bibliográficas sobre o tema, pouco estudado no Brasil