ABSTRACT
Fogo selvagem (FS) is a blistering skin disease caused by pathogenic IgG4 autoantibodies to desmoglein 1 (DSG1). Preclinical FS and leishmaniasis are endemic to certain regions of Brazil and exhibit nonpathogenic anti-DSG1 antibodies. Recurring bites from Lutzomyia longipalpis, the sand fly vector of leishmaniasis, immunize individuals with L. longipalpis salivary antigens LJM17 and LJM11. We measured the antibody responses to LJM17, LJM11, and DSG1 in normal settlers and patients with FS from an endemic focus of FS and nonendemic control populations. We also immunized mice with these antigens and assessed the IgG response. Healthy individuals and patients with FS from endemic areas had significantly higher values of IgG4 anti-LJM17 antibodies than nonendemic controls (P < 0.001 for both). The levels of IgG anti-DSG1 and IgG4 anti-LJM17 and anti-LJM11 antibodies correlated positively in normal settlers and patients with FS. Mice immunized with recombinant LJM17 produced IgG1 antibodies (human IgG4 homolog) that strongly cross-reacted with recombinant DSG1; these IgG1 antibodies were inhibited by LJM17, LJM11, and DSG1 in a dose-dependent manner. However, they did not bind human or mouse epidermis by indirect immunofluorescence. Lastly, we identified short-sequence homologies of surface-exposed residues within the human DSG1 ectodomain and LJM17. Inoculation by LJM17 from L. longipalpis-elicited DSG1-cross-reactive IgG4 antibodies may lead to FS in genetically predisposed individuals.
Subject(s)
Bites and Stings/immunology , Desmoglein 1/immunology , Insect Proteins/immunology , Pemphigus/immunology , Psychodidae/immunology , Animals , Autoantibodies/immunology , Autoantigens/immunology , Bites and Stings/epidemiology , Bites and Stings/pathology , Brazil/epidemiology , Cross Reactions , Disease Models, Animal , Endemic Diseases , Epidermis/immunology , Epidermis/pathology , Humans , Insect Vectors/immunology , Insect Vectors/parasitology , Leishmaniasis, Cutaneous/epidemiology , Leishmaniasis, Cutaneous/immunology , Leishmaniasis, Cutaneous/parasitology , Mice , Pemphigus/epidemiology , Pemphigus/pathology , Psychodidae/parasitology , Recombinant Proteins/immunology , Salivary Proteins and Peptides/immunologyABSTRACT
BACKGROUND: Investigators from Brasilia, Brazil, observed several patients with a mucocutaneous disease that resembles pemphigus vulgaris clinically and histologically but with epidemiological features of fogo selvagem. Our objective was to characterize antidesmoglein 3 and antidesmoglein 1 autoantibody profiles in these unique patients who reside in Goiânia and Brasilia, Brazil, known endemic regions of fogo selvagem. OBSERVATIONS: We performed serological evaluation of 8 patients with a mucocutaneous disease clinically and histologically consistent with pemphigus vulgaris, as well as 27 healthy relatives of patients with fogo selvagem who reside in these endemic areas. Serum samples from all 8 patients bound desmoglein 3 by cold immunoprecipitation and from 6 patients by enzyme-linked immunosorbent assay, while serum samples from 4 patients bound desmoglein 1 by cold immunoprecipitation and by enzyme-linked immunosorbent assay. Antidesmoglein 3 autoantibodies were detected in 4 of 27 healthy donors by cold immunoprecipitation and by enzyme-linked immunosorbent assay, whereas antidesmoglein 1 autoantibodies were detected in 6 individuals by cold immunoprecipitation and in 3 individuals by enzyme-linked immunosorbent assay. CONCLUSION: These findings provide serological evidence of a new endemic variant of pemphigus vulgaris.
Subject(s)
Autoantibodies/blood , Pemphigus/epidemiology , Adolescent , Adult , Brazil/epidemiology , Case-Control Studies , Desmoglein 1/immunology , Desmoglein 3/immunology , Endemic Diseases , Female , Humans , Male , Middle Aged , Pemphigus/blood , Pemphigus/etiology , Pemphigus/pathology , Seroepidemiologic StudiesABSTRACT
Canine hepatozoonosis is a disease caused by the tick-borne protozoan Hepatozoon spp. It has been reported in the United States, southern Europe, the Middle East, Africa and the Far East. In Brazil, canine hepatozoonosis is an emerging protozoal tick-borne disease, and is characterized by distinct clinical signs. The objective of this study was to analyze the laboratory findings of some hepatozoonosis cases in dogs in Brasília, Brazil, and their clinical signs. The animals of this experiment showed low parasitemia, similar to H. americanum, but the clinical signs presented were similar to H. canis. According to our observations and in agreement with O'Dwyer et al. [Vet. Parasitol. 94 (2001) 143], the Brazilian Hepatozoon appears more to resemble the species found in the eastern Hemisphere than with H. americanum of North America, or could be caused by a new species. Our data revealed that hepatozoonosis could be considered endemic in Brasília.
Subject(s)
Coccidia/growth & development , Coccidiosis/veterinary , Dog Diseases/parasitology , Animals , Brazil , Coccidiosis/blood , Coccidiosis/parasitology , Dogs , Erythrocyte Count/veterinary , Hematocrit/veterinary , Hemoglobins/metabolism , Leukocyte Count/veterinary , Lymphocyte Count/veterinaryABSTRACT
The Spitz nevus is a benign melanocytic lesion with clinical and histopathological features similar to those of melanoma. It was first described in 1948 but great controversy still remains today with respect to its diagnosis and management. The use of dermoscopy may increase diagnostic accuracy. In Spitz nevus, the most common dermoscopic finding is a starburst-like pattern, followed by globular and atypical patterns. Diagnosis must be confirmed by histopathology, particularly in atypical cases.
Subject(s)
Dermoscopy , Nevus, Epithelioid and Spindle Cell/pathology , Skin Neoplasms/pathology , Child, Preschool , Female , HumansABSTRACT
Tumor of the follicular infundibulum is an uncommon cutaneous lesion with different forms of clinical presentation, namely solitary and multiple/eruptive variants. The former shows predilection for head and neck and presents as a papulonodular scaly tumor. The latter is less frequent and occurs on facial, neck and upper chest areas as brown, reddish or more commonly hypopigmented macules leading to the differential diagnosis with vitiligo. The different clinical forms share the same histopathologic aspect. The present report describes a case of the multiple variant of tumor of the follicular infundibulum disclosing facial vitiligoid macules in a 35-year-old male patient.
Subject(s)
Hair Follicle , Hypopigmentation/pathology , Skin Neoplasms/pathology , Adult , Humans , Male , Vitiligo/pathologyABSTRACT
BACKGROUND: Endemic pemphigus foliaceus (EPF) or fogo selvagem (FS) is an endemic autoimmune disease, characterized by flaccid bullae induced by IgG(4) subclass antibodies. The authors demonstrate the epidemiological and clinical status of patients who have been followed at the University Hospital of Brasilia (HUB) for more than 15 years. METHODS: One hundred and ninety-six patients with FS took part in the project. In setting up this study a historical descriptive cohort of patients was put together. In order to collect data, the authors used a questionnaire where the patient indicated the sex, age at the onset, occupation, origin, clinical status, including scalp compromise, evolution, cofactors influencing clinical worsening and the treatment compliance. In order to minimize loss to follow up, the authors used the statistical method of incidence density (patients/years). RESULTS: The disease occurred in 58.4% of the young patients in the 11-30-year age bracket, and 52% came from urban areas. These patients included students and teachers. Localized disease predominated as compared with the generalized presentation of this condition. Fifty-nine percent of the patients evolved to the recurrent form. Those patients with the evolutional form, in remission for more than 1 year (94%), had been followed for more than 5 years. Even patients with the less active forms of the disease had scalp lesions. Thus scalp lesions are not an indicator of bad prognosis. CONCLUSIONS: In the present study, the disease affected patients from a higher sociocultural class than previously described. Furthermore, in contrast to other reports, a substantial number of the patients lived in urban areas, although often spending some time in a rural setting for leisure or professional activities. This study suggests that the longer the follow up, the higher the likelihood that the disease would progress to a more controlled clinical presentation. Scalp lesions were not related to adverse prognosis. Sun, heat and infections act as triggering factors for the immunological imbalance, worsening the disease.
Subject(s)
Pemphigus/epidemiology , Adolescent , Adult , Brazil , Child , Child, Preschool , Female , Hospitals, University , Humans , Infant , Male , Pemphigus/diagnosisABSTRACT
O nevo de Spitz é uma lesão melanocítica benigna com características clínicas e histopatológicas semelhantes às do melanoma. Foi descrito em 1948, mas até hoje, ainda, existe grande controvérsia no seu diagnóstico e conduta. A utilização da dermatoscopia pode aumentar a sua acurácia diagnóstica. As características dermatoscópicas do nevo de Spitz incluem um padrão estelar (starburst), que é o mais frequente, seguido do padrão globular e do padrão atípico. O diagnóstico deve ser confirmado por exame histopatológico, principalmente, nos casos atípicos.
The Spitz nevus is a benign melanocytic lesion with clinical and histopathological features similar to those of melanoma. It was first described in 1948 but great controversy still remains today with respect to its diagnosis and management. The use of dermoscopy may increase diagnostic accuracy. In Spitz nevus, the most common dermoscopic finding is a starburst-like pattern, followed by globular and atypical patterns. Diagnosis must be confirmed by histopathology, particularly in atypical cases.
Subject(s)
Child, Preschool , Female , Humans , Dermoscopy , Nevus, Epithelioid and Spindle Cell/pathology , Skin Neoplasms/pathologyABSTRACT
Tumor do infundíbulo folicular é lesão cutânea incomum, com várias formas de apresentação clínica. Destacam-se as formas solitária e eruptiva/múltipla. A primeira apresenta-se como lesão papulonodular descamativa em região de cabeça e pescoço. A segunda, alvo deste relato, é mais rara, ocorrendo em face, pescoço e porção superior do tronco como lesões maculares pardas, avermelhadas ou, mais frequentemente, hipopigmentadas, podendo levar ao diagnóstico diferencial com lesões vitiligóides. A apresentação microscópica das diferentes formas clínicas é similar. Neste artigo, relatamos um caso de tumor do infundíbulo folicular múltiplo/eruptivo apresentando-se como máculas vitiligóides faciais em homem de 35 anos.
Tumor of the follicular infundibulum is an uncommon cutaneous lesion with different forms of clinical presentation, namely solitary and multiple/eruptive variants. The former shows predilection for head and neck and presents as a papulonodular scaly tumor. The latter is less frequent and occurs on facial, neck and upper chest areas as brown, reddish or more commonly hypopigmented macules leading to the differential diagnosis with vitiligo. The different clinical forms share the same histopathologic aspect. The present report describes a case of the multiple variant of tumor of the follicular infundibulum disclosing facial vitiligoid macules in a 35-year-old male patient.
Subject(s)
Adult , Humans , Male , Hair Follicle , Hypopigmentation/pathology , Skin Neoplasms/pathology , Vitiligo/pathologySubject(s)
Angiomyoma/pathology , Palatal Neoplasms/pathology , Palate, Hard/pathology , Salivary Gland Neoplasms/pathology , Salivary Glands, Minor/pathology , Adenoma, Pleomorphic/pathology , Adenoma, Pleomorphic/surgery , Adult , Angiomyoma/surgery , Diagnosis, Differential , Female , Humans , Hyperplasia , Palatal Neoplasms/surgery , Palate, Hard/surgery , Salivary Gland Neoplasms/surgery , Salivary Glands, Minor/surgery , Telangiectasis/pathology , Telangiectasis/surgery , Treatment OutcomeABSTRACT
A síndrome do respirador bucal é caracterizado por alterações anatômicas que acarretam túrbios respiratórios induzindo as crianças afetadas a utilizarem a via bucal para respirar. Entre as alterações anatômicas que esses pacientes apresentam, está a típica fácies adenoidiana, caracterizando um indivíduo sempre com a boca aberta. tais alterações anatômicas conferem ao lábio inferior posição mais propensa à ação dos raios ultravioleta e das alterações actínicas decorrentes dessa exposição mais frequente. Os autores relatam o caso clínico de uma criança de 11 anos com síndrome do respirador bucal e alterações labiais intensas de quielite actínica. Defendem a idéia de que a síndrome do respirador bucal poderi ser um fator de risco a mais para o aparecimento da quielite actínica
Subject(s)
Humans , Child , Cheilitis , Mouth BreathingABSTRACT
O propósito da presente investigaçäo foi padronizar a reaçäo de imunofluorescência indireta para Pênfigo Foliáceo Endêmico (Fogo Selvagem). Verificamos que a pele humana normal é o substrato ideal e que pode proceder de prupúcio, cabeça, pescoço ou da parede abdominal anterior. A lavagem prévia da pele precedendo a incubaçäo com o soro deve ser evitada pois a antigenicidade pode ser diminuída. O TAS-cálcio preserva as propriedades antigênicas da pele e deve ser preferido como diluente para os soros. Lâminas cobertas com albumina säo úteis porque aumentam a aderência dos cortes de pele. A diluiçäo apropriada do conjugado é convenientemente determinada pelo teste de imunodifusäo radial (método de Ouchterlony). Com referência à correlaçäo entre título de anticorpos e atividade clínica, concluímos que um título igual ou maior do que 160 era de mau prognóstico pois estava asociado à forma generalizada da doença ou à casos de forma localizada refratários à terapêutica usual. Contudo, esta observaçäo requer confirmaçäo através de estudos que envolvam uma abordagem clínica apropriada
Subject(s)
Humans , Autoantibodies , Fluorescent Antibody Technique , Pemphigus/immunology , Immunodiffusion , Skin/immunologyABSTRACT
Os autores descrevem o resultado do inquérito dermatológico realizado entre os Xavánte da área Indígena Pimentel Barbosa, Mato Grosso, Brasil. Os resultados indicam a ocorrência de doenças relacionadas à higiene inadequada, como a escabiose, pediculose e piodermite. O encontro de perlèche sugere deficiência nutricional. O pênfigo foliáceo endêmico (fogo selvagem) foi a afecção dermatológica de maior gravidade presente nesta população
Subject(s)
Indians, South American , Skin Diseases , Pemphigus/epidemiologyABSTRACT
Foi realizado inquérito soro-epidemiológico através de testes de imunofluorescência indireta e imunoprecipitaçao visando determinar a prevalência do pênfigo foliáceo endêmico (PFE) na populaçao indígena Xavánte do Brasil Central. Do total de 163 amostras de soros testadas, 5 (3,1%) foram positivas à imunofluorescência. Todos os soros positivos procediam de indivíduos com sintomas clínicos da doença. Nao foram observadas diferenças do PFE entre os Xavánte e a populaçao em geral sob o ponto de vista imunológico, já que se verificou uma predominância de IgG4 entre os anticorpos nos pacientes indígenas
Subject(s)
Fluorescent Antibody Technique , Indians, South American , Pemphigus/epidemiologyABSTRACT
Penfigóide bolhoso (PB) é raro na criança e no adulto jovem. Relatamos aqui o caso de um paciente de 22 anos que apresentou erupçäo bolhosa com características clínicas e imunopatológicas de PB induzido por medicaçäo antigripal (ácido acetil-salicílico + cafeína). Näo existem relatos na literatura de PB provocado por essa associaçäo medicamentosa. Säo feitas consideraçöes sobre a participaçäo de drogas no desencadeamento de PB possíveis associaçöes com doenças auto-imunes
Subject(s)
Humans , Male , Female , Adult , Middle Aged , Aspirin/adverse effects , Pemphigoid, Bullous/chemically induced , Prednisone/therapeutic use , Brazil , Diagnosis, Differential , Pemphigoid, Bullous/diagnosis , Pemphigoid, Bullous/drug therapy , Pemphigoid, Bullous/etiologyABSTRACT
A fasciite eosinofílica é síndrome de etiologia desconhecida, descrita em 1974, caracterizada por rigidez de pele secundária a fasciite difusa, eosinofilia periférica e hipergamaglobulinemia. Embora similar em muitos aspectos à esclerodermia, apresente características clínicas, laboratoriais e histopatológicas próprias. A resposta terapêutica e a evoluçäo também diferem das da esclerodermia. As características da fasciite eosinofílica, seu diagnóstico diferencial e prognóstico säo discutidos na presente revisäo
Subject(s)
Humans , Eosinophilia/diagnosis , Fasciitis/diagnosis , Adrenal Cortex Hormones/therapeutic use , Diagnosis, Differential , Eosinophilia/drug therapy , Fasciitis/drug therapy , Scleroderma, Localized/diagnosisABSTRACT
Os autores relatam o caso de uma paciente de 65 anos que há três referia telangiectasias e máculas eritematosas disseminadas pela região torácica anterior, abdômen e membros inferiores, associadas a prurido eventual sem sintomas sistêmicos. No exame histopatológico foram demonstrados ectasia vascular e mastócitos pelo azul de toluidina. Foi feito o diagnóstico de Telangiectasia macularis eruptiva perstans. Trata-se de forma rara de mastocitose, geralmente de difícil diagnóstico e refratária ao tratamento
Subject(s)
Humans , Female , Aged , Mastocytosis , TelangiectasisABSTRACT
É descrito um caso de linfadenite necrosante sem infiltraçäo granulocítica (doença de Kikuchi). Aspectos clínicos e laboratoriais da doença säo discutidos, assim como seu diagnóstico diferencial com outras linfadenopatias
Subject(s)
Adult , Humans , Male , Lymphadenitis/pathologyABSTRACT
Os autores relatam um caso de penfigóide gestacional iniciado no quito mês de gestaçäo, confirmado por meio de técnica de imunofluorescência e com boa resposta à corticoterapia. Fazem breve revisäo da literatura e chamam a atençäo para a substituiçäo da terminologia herpes gestacional por penfigóide gestacional.
Subject(s)
Humans , Female , Pregnancy , Adult , Administration, Topical , Dexamethasone/administration & dosage , Pemphigoid Gestationis/diagnosis , Pemphigoid Gestationis/drug therapy , Pemphigoid Gestationis/therapy , Prednisone/therapeutic use , Pregnancy Complications , Fluorescent Antibody Technique , Pregnancy ComplicationsABSTRACT
O fogo selvagem (FS) apresenta características clinicoepidemiológicas peculiares. O histopatológico, a imunofluorescência direta e a indireta são os exames mais utilizados para diagnóstico da doença. O objetivo era analisar os achados da imunofluorescência direta (IFD) e indireta (IFI) dos pacientes de fogo selvagem (FS) do Hospital Universitário de Brasília (HUB). De 176 pacientes portadores de FS diagnosticados no HUB foram coletados os resultados da histopatologia, IFD e IFI no período de março de 1985 a março de 1999. Os títutlos de IFI foram correlacionados com o número de lesões cutâneas. A histopatologia mostrou acantólise subcórnea em 88 por cento. As IFDs foram positivas para IgG em 88 por cento e para C3 em 54 por cento. Percentual que variou de 90 a 95 por cento dos pacientes sem lesões ou com até seis lesões apresentavam títulos de IFI até 1/160. A IFI contribuiu para a valiar a atividade da doença, bem como para seu diagnóstico, o complementeo parece colaborar para o desencadeamento das lesões
Subject(s)
Humans , Fluorescent Antibody Technique , PemphigusABSTRACT
Os autores relatam um caso de penfigóide bolhoso vulvar localizado da criança corroborado pelo exame de imunofluorescência indireta (técnica de salt-split). Fazem breve revisão da literatura chamando a atenção para a raridade do caso e destacam a importância do diagnóstico diferencial