Sebastiäo A. P Sampaio

Endemic pemphigus foliaceus (fogo seivagem). 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.

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<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<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.

Brazilian pemphigus foliaceus

Abstract Fogo Selvagem (Wildfire) is a cutaneous disease characterized by a specific epidemiology, generalized distribution of bullae followed by an exfoliative erythroderma, and frequently, a grave prognosis, if untreated. Its etiology is unknown and the pathogenesis only partly understood. The histopathologic hallmark is the presence of acantholysis in the superficial layers of the epidermis. The acantholysis is due to the binding of immunoglobulin to the intercellular cement substance of the epidermis.

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.

Endemic pemphigus foliaceus in western Paraná, Brazil (1976-1988).

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.

Pemphigus foliaceus autoantibodies bind both epidermis and squamous mucosal epithelium, but tissue injury is detected only in the epidermis

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.

Elevated thymosin alpha I levels in Brazilian pemphigus foliaceus.

Levels of thymosin alpha I in the sera of 37 patients with Brazilian pemphigus foliaceus (BPF) were measured using a competitive binding radioimmunoassay. The values were compared with 19 patients with other forms of pemphigus, 13 relatives of patients with BPF, 18 patients with other dermatological diseases, and 265 normal controls. We found that 27 (73%) of the patients with BPF had thymosin alpha I serum levels that were at least two standard deviations above the mean for normal individuals. The mean value for patients with BPF was significantly greater than any other groups studied. The thymosin elevation is similar to alterations seen in certain viral diseases and suggests that BPF is aetiopathogenically distinct from the other forms of pemphigus.

The treatment of Brazilian pemphigus foliaceus (fogo selvagem).

Brazilian pemphigus foliaceus (fogo selvagem) occurs endemically in the central-southern states, especially in the areas that are still being settled. Before corticoids were introduced into the treatment of this dermatosis, prognosis was poor. Tlie ailment took either an acute or chronic form, frequently being fatal. In some exceptional cases the ailment subsided spontaneously. With the goal of studying therapeutic results as well as side ellects of corticoid therapy for this condition, a work group was formed that carried out therapeutic tests with thiee corticoids in four diflerent centers: Sao Paulo (SP) Goiania (CO), Belo Horizonte (MG) and Rio de Janeiro (GB). Experiments were carried out simultaneously and the participants Iield regular meetings during this twelve-month period.

Remissão de lentigo maligno extenso após tratamento com imiquimod

Lentigno maligna is a melanoma in situ that most commonly appears on areas exposed to ultraviolet radiation, in elderly patients. Treatment is required mainly to minimize the risk of progression to lentigo maligna melanoma. The present report refers to an elderly patient with recurrent lesions of lentigo maligna in her face, who was successfully treated with topical imiquimod, which showed to be a useful therapy for some cases of the disease.

The Ultrastructural and Histophotometric Study of Elastic and Collagen Fibers in Type II Ehlers-Danlos Syndrome and Subclinical Forms

Skin disorders in type II Ehlers-Danlos Syndrome (EDS) are characterized by signs of cutaneous hyperdistensibility, skin and vascular fragility, atrophic scars, and articular hypermobility. These features may have less important clinical presentation in the intermediate forms of type II EDS. The authors studied the ultrastructural and quantitative aspects of elastic and collagen fibers in the skin of individuals with subclinical signs of type II of Ehlers-Danlos Syndrome. A group of 27 individuals (Group I) with large atrophic scars, articular hypermobility of the hands, and cutaneous and vascular fragility were compared with 10 healthy individuals. The subjects from both groups were volunteers from Hospital das Clínicas da Universidade de São Paulo. The elastic fibers did not show alterations but collagen ultrastructural abnormalities were seen in diameter and curvature, such as torsion, collagen flower-like aspect and discrete mass enlargement by histophotometry.Skin disorders in type II Ehlers-Danlos Syndrome (EDS) are characterized by signs of cutaneous hyperdistensibility, skin and vascular fragility, atrophic scars, and articular hypermobility. These features may have less important clinical presentation in the intermediate forms of type II EDS. The authors studied the ultrastructural and quantitative aspects of elastic and collagen fibers in the skin of individuals with subclinical signs of type II of Ehlers-Danlos Syndrome. A group of 27 individuals (Group I) with large atrophic scars, articular hypermobility of the hands, and cutaneous and vascular fragility were compared with 10 healthy individuals. The subjects from both groups were volunteers from Hospital das Clínicas da Universidade de São Paulo. The elastic fibers did not show alterations but collagen ultrastructural abnormalities were seen in diameter and curvature, such as torsion, collagen flower-like aspect and discrete mass enlargement by histophotometry.