Quality of life is severely compromised in adult patients with atopic dermatitis in Brazil, especially due to mental components.

PURPOSE: to measure the quality of life (QoL), either by a specific dermatology or generic self applied questionnaire, in Atopic dermatitis adult Brazilian patients, looking for selected affected groups. METHODS: We studied the quality of life of 75 Brazilian ambulatory adults with atopic dermatitis using two types of self-answered instruments: a quality of life generic questionnaire (SF-36) and a 10-item Dermatology Life Quality Index (DLQI) questionnaire. All patients had been treated for at least 6 months, and their disease status was determined by Eczema Area and Severity Index scores. RESULTS: Quality of life and disease control were found to be related but with low scores both in DLQI (r(2)=0.26) and in SF-36 (r(2)=0.20), but with greater correlation for SF-36 mental components. Using the 75% percentile distribution of SF36 mean score and the 75% value of disease severity score, we sorted patients into four groups: I, referring good QoL and mild atopic disease (14/75), II, referring bad QoL and with mild atopic disease (19/75), III referring good QoL despite severe atopic disease (5/75) and IV referring bad QoL and severe atopic disease (37/75); all groups presented similar age, education, family income and time of disease progression. There was a higher frequency of women in group II, but without sleep disturbance or increased pruritus, which was present in group IV, with intense itching and sleep disturbances. Analyzing the physical or mental components of the SF36 generic test, discrepant groups II and III presented higher differences related to the mental components of the test, which was also related to DLQI scores, with a similar distribution for the 2 groups and a higher relation to the mental component of the generic test. CONCLUSION: The quality of life is affected in adult atopic patients, both related to disease severity and also to mental components, but with diverse effects in patient subgroups. Our data show some components that may mask the exact relationship between QoL results and disease severity.

Quality of life is severely compromised in adult patients with atopic dermatitis in Brazil, especially due to mental components

PURPOSE: to measure the quality of life (QoL), either by a specific dermatology or generic self applied questionnaire, in Atopic dermatitis adult Brazilian patients, looking for selected affected groups. METHODS: We studied the quality of life of 75 Brazilian ambulatory adults with atopic dermatitis using two types of self-answered instruments: a quality of life generic questionnaire (SF-36) and a 10-item Dermatology Life Quality Index (DLQI) questionnaire. All patients had been treated for at least 6 months, and their disease status was determined by Eczema Area and Severity Index scores. RESULTS: Quality of life and disease control were found to be related but with low scores both in DLQI (r²=0.26) and in SF-36 (r²=0.20), but with greater correlation for SF-36 mental components. Using the 75 percent percentile distribution of SF36 mean score and the 75 percent value of disease severity score, we sorted patients into four groups: I, referring good QoL and mild atopic disease (14/75), II, referring bad QoL and with mild atopic disease (19/75), III referring good QoL despite severe atopic disease (5/75) and IV referring bad QoL and severe atopic disease (37/75); all groups presented similar age, education, family income and time of disease progression. There was a higher frequency of women in group II, but without sleep disturbance or increased pruritus, which was present in group IV, with intense itching and sleep disturbances. Analyzing the physical or mental components of the SF36 generic test, discrepant groups II and III presented higher differences related to the mental components of the test, which was also related to DLQI scores, with a similar distribution for the 2 groups and a higher relation to the mental component of the generic test. CONCLUSION: The quality of life is affected in adult atopic patients, both related to disease severity and also to mental components, but with diverse effects in patient subgroups. Our data show...

OBJETIVOS: Medir a qualidade de vida (QoL), por questionários tanto genéricos como dermatológicos específicos, em pacientes adultos brasileiros com dermatite atópica, procurando por grupos afetados selecionados. MÉTODOS: Nós estudamos a qualidade de vida em 75 pacientes brasileiros adultos em tratamento ambulatorial de dermatite atópica, usando dois tipos de questionários de auto-resposta, previamente padronizados: um questionário genérico de qualidade de vida de 36 questões (SF-36) e um questionário de 10 questões para determinação do índice dermatológico de qualidade de vida (DLQI). Todos os pacientes estavam em tratamento por pelo menos seis meses e o seu estágio de doença clinico definido quantitativamente pelo índice padronizado de gravidade e áreas de eczema (EASI). RESULTADOS: A QoL e o controle da doença estavam relacionados mas com baixa relação tanto avaliados pelo DLQI (r²=0.26) ou pelo SF-36 (r²=0.20), mas com maiores relações com os componentes emocionais do SF-36. Usando a distribuição do percentil 75 por cento para o SF36 e os valores de 75 por cento do escore de gravidade clínica EASI, os pacientes foram distribuídos em quatro grupos: I que referia boa QoL e doença atópica leve (14/75), II referindo má QoL e doença atópica leve (19/75), III referindo boa QoL apesar de doença atópica mais grave (5/75) e IV concordando uma má QoL referida e uma doença atópica mais grave (37/75); todos os grupos apresentavam mesma distribuição etária, educação e nível social, renda familiar e tempo de doença. Havia uma maior freqüência de mulheres no grupo II, mas sem distúrbios de sono ou prurido intenso, fatores presentes intensamente no grupo IV. Dissecando os componentes físicos e emocionais do SF36, os grupos discrepantes II e II apresentavam maior diferença relativa aos componentes emocionais do teste, que também estavam relacionados aos valores do teste DLQI, o qual apresentava uma distribuição similar entre os grupos e maior relação...

Clinical and serological follow-up studies of endemic pemphigus foliaceus (fogo selvagem) in Western Parana, Brazil (2001-2002).

BACKGROUND: Fogo selvagem (FS) has been described in several regions of Brazil, including the Western regions of the state of Parana. In 1990, Empinotti et al. reported case studies of 213 patients with FS that were collected from 1976 to 1988. The same author (J.C.E.) has observed that the frequency of cases in these regions of Parana has decreased. OBJECTIVES: The purpose of this study was to clinically and serologically evaluate a small group of the patients originally reported in 1990 and compare data with a group of control individuals. These patients were treated at the onset of the disease with systemic steroids. PATIENTS AND METHODS: Patients with FS, their unaffected relatives (n = 80) and genetically unrelated controls (n = 15) were identified during a field study from 1 May 2001 to 30 June 2002. Sera from nine patients with FS and six normal controls that were collected in the 1976-1988 evaluation were available for this study. The sera were tested by indirect immunofluorescence, enzyme-linked immunosorbent assay (ELISA) and immunoprecipitation using recombinant human desmoglein 1 (Dsg1). RESULTS: Only 16 of the originally identified 213 patients with FS were found during the field studies. Thirteen of the 16 patients were in clinical and serological remission; 20% of normal controls (19 of 95) were positive in the Dsg1 ELISA. The majority of these subjects (17 of 19) were genetically related to FS patients. Six normal controls that were positive in the Dsg1 ELISA in the original survey were found to be negative or weakly positive in this evaluation. CONCLUSION: The reduced frequency of positive serological markers of disease in patients and normal controls from Western Parana, as well as the absence of recurrent disease in previously identified patients, suggest that environmental antigenic stimulation of the population at risk may have decreased in recent years.

Comparison of black fly species (Diptera: Simuliidae) on an Amerindian reservation with a high prevalence of fogo selvagem to neighboring disease-free sites in the State of Mato Grosso do Sul, Brazil. The Cooperative Group on Fogo Selvagem Research.

Fogo selvagem is an autoimmune blistering skin disease that principally occurs among rural Brazilians living in geographically clumped disease foci. Exposure to hematophagous black flies possibly is related to the cause of the disease. We compared the occurrence, proportions, and richness of simuliid species immatures and the biting activity of adult females within a recently discovered, high prevalence focus of fogo selvagem, the Limão Verde Terena Reservation, to that of neighboring regions with no reported cases of fogo selvagem. Nine black fly species were collected from 12 stream sites during 5 trips to the fogo selvagem focus. The species showed longitudinal (upstream-downstream) trends in occurrence, proportions, and richness, and the abundance of simuliid immatures was greater at downstream sites. The most prevalent species at the focus, Simulium nigrimanum (Macquart), dominated the stream sites with highly abundant simuliid assemblages, and was the most common black fly in human bait collections. This species was absent or in very low numbers in neighboring valleys and villages that did not have cases of fogo selvagem.

Safety evaluation of chronic fluconazole therapy. Fluconazole Pan-American Study Group.

The possible adverse effects of chronic, high-dose fluconazole therapy are detailed from analysis of a multicenter, dose-escalating study of the therapy of invasive mycoses. Ninety-three adult patients were studied, 48 of these received > or = 6 months therapy and 20 received > or = 1 year. Fifty-eight patients received > or = 300 mg/day, and 7 received > or = 600 mg/day. One patient received 1,997 g over 86 months. Twenty-seven percent experienced possible symptomatic side effects, which resulted in 2 patients discontinuing therapy, and 42% had asymptomatic laboratory abnormalities, none of which were progressive. Headache, hair loss and anorexia were the most common symptoms experienced (each by 3% of patients), and eosinophilia and aspartate aminotransferase increases were the most common laboratory findings (12 and 10%, respectively). Fluconazole appears well tolerated and safe in these doses and durations.

Estudo multicêntrico comparando a eficácia e tolerância da isotretinoina gel 0,05 por cento e tretinoina creme 0, 005 por cento no tratamento da acne vulgar / A multicenter trial comparing the efficacy and tolerance of isotretinoin gel 0, 05 por cento and tretinoin cream 0, 05 por cento in the treatment of acne vulgaris

A isotretinoina tópica tem se mostrado efetiva no tratamento de formas leves e moderadas da acne vulgar. Objetivo da investigaçäo: Comparar a eficácia e tolerância da isotretinoina gel 0, 05 por cento com a tretinoina creme 0, 05 por cento no tratamento de formas leves e moderadas da acne vulgar.Método: Foram tratados 28 pacientes com acne vulgar de ambos os sexos, idade de 13 a 30 anos, com 15 a 100 lesöes inflamatórias e näo inflamatórias na face e nao mais que três lesöes nódulo-cisticas. Os pacientes foram distribuidos aleatoriamente em dois grupos, cada paciente fazendo uma aplicaçäo diária de isotretinoína gel 0, 05 por cento ou tretinoína creme 0, 05 por cento em toda face por dose semanas. A eficácia foi determinada objetivamente pelo decréscimo no número de lesöes e avaliada subjetivamente no final do tratamento pelos investigadores e pacientes. A tolerância foi avaliada durante o tratamento pelo aparecimento de ardor, prurido, descamaçäo, secura e eritema. No final do tratamento investigadores e pacientes avaliaram globalmente a tolerância. Resultado: Observou-se decrécimo de número de lesöes inflamatórias e näo-inflamatórias, nos dois grupos, sem diferença estatisticamente significante. Assim, isotretinoína gel 0, 05 por cento e tretinoína creme 0, 05 por cento mostraram açäo terapêutica similar. Em relaçäo a tolerância, verificou-se melhor tolerância à isotretinoína, com menor frequência de reaçöes adversas, estatisticamente significante, em comparaçäo a tretinoína. Conclusäo: Isotretinoína gel 0, 05 por cento tem açäo terapêutica similar à tretinoína creme 0, 05 por cento, porém é melhor tolerada

An active focus of high prevalence of fogo selvagem on an Amerindian reservation in Brazil. Cooperative Group on Fogo Selvagem Research.

Fogo Selvagem (FS) is an autoimmune disease characterized by subcorneal vesicles and antidesmoglein-1 autoantibodies. Previous epidemiologic data have linked the onset of FS to exposure to an environmental antigen(s). This investigation describes a unique human settlement with an extraordinarily high prevalence of FS. This community is made up of Amerindians belonging to the Terena tribe, which has settled on the Limao Verde reservation in the state of Mato Grosso do Sul in Brazil. Twenty-six well-characterized FS cases have been identified within a total population of 998, yielding a prevalence of 2.6%. Seventeen of the patients (65 %) were males, and over 50% were older than 30 y of age. The incidence of the disease shows temporal periodicity, i.e., years with several cases of FS alternating with years with no cases. Over one-half of the cases occurred in genetically related family members. Another Terena reservation, the Ipegue/Taunay, located 90 km west of the Limao Verde reservation, was also evaluated as a control group. This reservation, with a population of 2203, had no recorded cases of FS. Thus, the Limao Verde reservation represents a new focus of FS in which the disease exhibits temporal, geographic, and familial clustering. These results suggest that the environmental antigen or antigens precipitating FS are endemic to the Limao Verde reservation. This reservation appears to be an ideal population for carrying out sero-epidemiologic, genetic, and environmental studies aimed at disclosing the etiology of FS.

Pemphigus foliaceus autoantibodies bind both epidermis and squamous mucosal epithelium, but tissue injury is detected only in the epidermis. The Cooperative Group on Fogo Selvagem Research.

BACKGROUND: Fogo selvagem (FS) is an autoimmune intraepidermal blistering disease mediated by antidesmosomal autoantibodies. Patients with FS do not have mucosal lesions despite extensive skin involvement. OBJECTIVE: Our purpose was to evaluate the epidermis and the oral epithelium of patients with FS as targets of antidesmosomal autoantibodies. METHODS: Fifteen patients were studied clinically, histologically, and immunologically. Biopsy specimens from the skin and the oral mucosa were studied by light microscopy and direct immunofluorescence. The serum of each of these patients was also titrated by indirect immunofluorescence. RESULTS: All patients showed skin lesions and subcorneal acantholyis, but none exhibited oral blisters or erosions. Direct immunofluorescence analysis demonstrated the presence of tissue-bound autoantibodies in both the epidermis and the oral epithelium of all patients with FS. Antiepidermal autoantibodies were also found in the sera of the patients. CONCLUSION: Relevant epitopes on desmoglein 1 molecules of oral epithelium may not be available to react with pathogenic FS autoantibodies.

Brazilian pemphigus foliaceus, endemic pemphigus foliaceus, or fogo selvagem (wild fire).

Brazilian pemphigus foliaceus, also known as fogo selvagem, is the endemic form of pemphigus foliaceus occurring in certain regions of Brazil. Epidemiologic data strongly support the notion that this disease is caused by exposure to an environmental agent or agents. This form of pemphigus foliaceus is a true human autoimmune disease mediated by autoantibodies of the IgG class, IgG4 subclass. These autoantibodies are pathogenic and highly specific for the disease. The environment agent may sensitize the patient to produce autoantibodies. These autoantibodies may cross-react with the epidermis and induce disease in the patient.

Successful treatment of sporotrichosis with oral fluconazole: a report of three cases.

We report three cases of sporotrichosis successfully treated with oral fluconazole. A verrucous lesion on the toe was cured after 126 days, and a lesion on the left foot resolved after 91 days' treatment. A case of lymphangitic-type sporotrichosis required 174 days of treatment to achieve a cure, and a higher dose (400 mg daily) was necessary in this case. Any side-effects were insignificant. We conclude that this new bis-triazole compound can be successfully used as an alternative treatment for sporotrichosis when conventional drugs must be avoided.

Situação de trabalho de médicos egressos de programas de residência médica no Estado de São Paulo / Work situation of physicians graduating from medical residency programs in the State of São Paulo, Brazil

The authors report on a study conducted to analyze the professional situation of physicians residing in the State of São Paulo, Brazil, five years after completion of a medical residency program. At least 50 of the professionals were interviewed in each specialty. A heavy work load was observed in physicians working both in the metropolitan area of the State capital and in other regions of the State. Male doctors had a higher income than female ones. The latter, however, had a higher proportion working in a single specialty, although 16.8 did not practice in the specialty in which they were trained. The most frequent working environment was a hospital, followed by an ambulatory facility and a private office. The metropolitan area physicians exceeded the remaining in teaching activities and public hospital appointments. They had also a higher tendency to work in a different specialty. This study offers significant information with respect to professional decisions as well as regarding the creation or expansion of residency programs.

[Work situation of physicians graduating from medical residency programs in the State of São Paulo, Brazil]. / Situação de trabalho de médicos egressos de programas de residência médica no Estado de São Paulo.

The authors report on a study conducted to analyze the professional situation of physicians residing in the State of São Paulo, Brazil, five years after completion of a medical residency program. At least 50% of the professionals were interviewed in each specialty. A heavy work load was observed in physicians working both in the metropolitan area of the State capital and in other regions of the State. Male doctors had a higher income than female ones. The latter, however, had a higher proportion working in a single specialty, although 16.8% did not practice in the specialty in which they were trained. The most frequent working environment was a hospital, followed by an ambulatory facility and a private office. The metropolitan area physicians exceeded the remaining in teaching activities and public hospital appointments. They had also a higher tendency to work in a different specialty. This study offers significant information with respect to professional decisions as well as regarding the creation or expansion of residency programs.

Environmental risk factors in endemic pemphigus foliaceus (Fogo selvagem). "The Cooperative Group on Fogo Selvagem Research".

Endemic pemphigus foliaceus or Fogo selvagem (FS) is an epidermal organ-specific autoimmune disease mediated by autoantibodies. Individuals at risk are peasants who live and work on farms located in the interior of certain endemic states of Brazil. This case-control study compares a group of 52 FS patients with 52 patients suffering from other dermatoses admitted and followed at the hospital for pemphigus (Hospital do Penfigo) in the city of Goiania, state of Goias. Patients and controls matched 1:1 by age, sex, and occupation were examined by two dermatologists at the time of admission and asked to respond to a prepared questionnaire. This questionnaire concerned current and past (1 and 5 years) exposure to environmental risk factors. The following risk factors were assessed: black fly bites, presence of rodents at home, exposure to cereal dust, exposure to fumes or dust released by tree and shrub removal, and exposure to insecticides. Relative risks were estimated from tabulated data by the odds ratio and tested for significance by the chi-square test. The 95% confidence interval for the odds ratio was also calculated for each of the risk factors. The only risk factor showing an odds ratio significantly different from one was exposure to simuliidae bites (odds ratio 4.7, p less than 0.001). This study reinforces the hypothesis that chronic exposure to black fly antigens may precipitate IgG4 antibody formation in predisposed individuals. These antibodies in turn may cross-react with epidermal antigens and cause acantholysis and the clinical expression of the disease known as FS.

Cutaneous lupus erythematosus: direct immunofluorescence and epidermal basal membrane study.

Biopsy specimens of cutaneous discoid lesions of 71 patients with cutaneous lupus erythematosus (CLE) were studied. The material was examined by direct immunofluorescence (DIF) to establish positivity and morphologic patterns of immunoglobulins and complement deposits in the basement membrane zone (BMZ). A correlation between DIF results and thickening of the epidermis basement membrane (BM) stained by periodic acid-Schiff (PAS), obtained from 31 matched biopsy specimens, also is presented. Direct immunofluorescence had positive results in 66.20% of the 71 examinations and 70.97% of the matched examinations, whereas PAS showed BM thickening in 100% of the specimens. This observation stresses the importance of such histopathologic findings in the diagnosis of discoid lesions in CLE. There was no correlation between DIF patterns and PAS-stained BMZ thickening. These findings merit additional study. In conclusion, an accordance between PAS-stained BM thickening and immunoglobulin deposits has occurred in 70.97% of cases. This phenomenon does not depend on the presence of immunocomplexes, for it occurs even in cases in which immunocomplex deposits were not detected.

A Pan-American 5-year study of fluconazole therapy for deep mycoses in the immunocompetent host. Pan-American Study Group.

Eighty-eight immunocompetent patients with deep mycoses from eight countries were evaluated with the same protocol for efficacy of fluconazole monotherapy. Entry doses were raised from 100 to 400 mg as safety was shown in initial cohorts, and dosages up to 2,400 mg daily and durations up to 44 months were studied. Results were very similar in different countries. Twenty-seven of 28 evaluable patients with paracoccidioidomycosis, 13 of 19 with sporotrichosis, 14 of 16 with coccidioidomycosis, and eight of eight with histoplasmosis demonstrated objective responses to therapy, as did one patient each with zygomycosis and alternariosis. For these patients, relapses have been unusual thus far. In contrast, one patient with chromoblastomycosis responded but relapsed, and six did not respond; one patient with mycetoma responded but relapsed, and two did not respond. The drug was well tolerated by patients, including six who received intravenous therapy. In vitro susceptibility tests suggested that clinical response was correlated with susceptibility but that resistance did not preclude clinical response. Fluconazole therapy appears efficacious for several deep mycoses; dosages of greater than 200 mg daily may be needed for some diseases. The further evaluation of fluconazole for these entities is warranted.

HLA antigens and risk for development of pemphigus foliaceus (fogo selvagem) in endemic areas of Brazil.

Endemic pemphigus foliaceus (EPF), is an autoimmune disease associated with production of IgG antibodies against epidermal antigens. We have tested 38 patients and 50 control subjects living in endemic areas to investigate whether HLA genes are associated with host factors that determine whether or not exposed individuals will develop this disease. A variant of HLA-DR1, an antigen common in Blacks (DRB1*0102), was found to be the main susceptibility factor (relative risk = 7.3, P less than 0.0002). Two amino acids, in positions 85 and 86 of DRB1, distinguish DRB1*0102 from DRB1*0101. These residues appear to be involved in the formation of a functional epitope that causes T cell recognition and determines disease susceptibility. Moreover, subjects having DQw2 did not develop the disease, while the frequency of DQw2 in controls was 22% (RR = 0.04, P less than 0.006). Thus HLA genes appear to play a crucial role in the response to an environmental factor which in this setting frequently leads to the development of autoimmune disease. An HLA-DQ allele, DQw2, appears to be associated with factors that prevent the development of the disease in exposed individuals.

Endemic pemphigus foliaceus in western Parana, Brazil (1976-1988). Cooperative Group for Fogo Selvagem Research.

Endemic pemphigus foliaceus or fogo selvagem (FS) is a blistering autoimmune disease indigenous to certain states of Brazil. In the state of Parana the disease has been reported in the north-central regions where a total of 632 cases were documented in the period of 1940-80. The present study describes a new focus of FS in the western region of the state of Parana. This focus includes a total of 213 new cases of FS and only 11 cases of pemphigus vulgaris seen in this region from February 1976 to July 1988. Over 90% of these patients were peasants working in agriculture or involved in other outdoor activities.

Endemic pemphigus foliaceus (fogo selvagem). I. Clinical features and immunopathology.

Endemic pemphigus foliaceus is an autoimmune disease that has remarkable features. Endemic foci are found in characteristic environments within the interior of Brazil. The epidemiologic data strongly suggest that an environmental factor initiates the autoantibody response in the host. As such it is an important disease for in-depth study. A group of interested investigators in both Brazil and the United States has been formed to attempt to do just that. As part of the overall effort, this Cooperative Research Group for the Study of Fogo Selvagem presents a definition of the disease, a proposed clinical classification for various forms of the disease, and an outline of what is currently known of its immunopathologic characteristics.

Endemic pemphigus foliaceus (Fogo Selvagem): II. Current and historic epidemiologic studies.

This paper details current and historic epidemiologic features of Fogo Selvagem (Endemic pemphigus foliaceus) in Brazil. The following features are described. a) The disease occurs in endemic fashion in regions of Brazil within the states of Goias, Mato Grosso do Sul, Parana, Sao Paulo, and Minas Gerais. It appears that the disease is spreading toward the northwest and west, involving the states of Mato Grosso, Para, Maranhao, Rondonia, Acre, and Amazonas. b) People at risk are young peasants or children of either sex or any race exposed to the local ecology in rural areas of endemic states. Although the disease has been described in urban centers, these occurrences are rare. c) Fogo Selvagem commonly appears in wild areas being colonized and disappears as these areas become urbanized. d) The majority of patients live in close proximity to rivers and within the 10-15 Km flying range of mosquitos or black flies (such as Simulium). It is hypothesized that a black fly, Simulium pruinosum may be the vector that precipitates the disease. f) There is a significant number of Fogo Selvagem in family units where multiple, genetically related individuals are affected. g) Finally, autoantibodies against lupus-associated antigens are not present in the sera of patients with Fogo Selvagem. Clinical examination of the skin, and serologic screening for pemphigus autoantibodies are specific parameters that can be used in the search for the etiologic agents that lead to autoimmune disease of the skin. To identify and prove an etiologic agent for this well-characterized autoimmune disease would be of tremendous importance to the understanding of autoimmune skin diseases, and potentially other organ-specific autoimmune disorders.

Treatment of chromomycosis by cryosurgery with liquid nitrogen: a report on eleven cases.

Cryosurgery with liquid nitrogen was used to treat 11 cases of chromomycosis, 5 with localized lesions and 6 with generalized ones. Freezing time varied from 30 seconds to 4 minutes and the number of cycles from 1 to more than 40. All 5 cases with localized lesions responded extremely well to treatment, with no relapse for up to 53 months. Three patients with generalized lesions attained clinical and mycologic remission for up to 26 months, while 3 had significant improvement without cure. Cryosurgery with liquid nitrogen is therefore an effective treatment for chromomycosis, especially for cases presenting with localized lesions.