RESUMO
The authors present an uncommon case of systemic amyloidosis associated with multiple myeloma in a 35-year old woman. Systemic amyloidosis commonly presents in association with clonal plasma cell proliferative disorders, and less frequently as secondary or of a hereditary origin. Amyloidosis is usually associated with multiple myeloma in older patients and frequently has an unfavourable prognosis.
Los autores presentan aquí un caso raro de amiloidosis sistémica asociado con mieloma múltiple en una mujer de 35 años de edad. La amiloidosis sistémica normalmente se presenta asociada con desórdenes proliferativos de las células plasmáticas clonales, y con menor frecuencia con origen secundario o hereditario. Amiloidosis se asocia normalmente con el mieloma múltiple en pacientes de mayor edad y frecuentemente tiene una prognosis desfavorable.
Assuntos
Adulto , Feminino , Humanos , Amiloidose/complicações , Cadeias Leves de Imunoglobulina/metabolismo , Mieloma Múltiplo/complicações , Amiloidose/metabolismoRESUMO
The authors present an uncommon case of systemic amyloidosis associated with multiple myeloma in a 35-year old woman. Systemic amyloidosis commonly presents in association with clonal plasma cell proliferative disorders, and less frequently as secondary or of a hereditary origin. Amyloidosis is usually associated with multiple myeloma in older patients and frequently has an unfavourable prognosis.
Assuntos
Amiloidose/complicações , Cadeias Leves de Imunoglobulina/metabolismo , Mieloma Múltiplo/complicações , Adulto , Amiloidose/metabolismo , Feminino , HumanosRESUMO
BACKGROUND: Pemphigus foliaceus is an autoimmune skin disease mediated by autoantibodies against desmoglein 1. The endemic form is thought to have an environmental cause. The Terena reservation of Limão Verde in Mato Grosso do Sul, Brazil, is a recently identified focus of the disease, with a prevalence of 3.4 percent in the population. We tested the hypothesis that normal subjects living in an endemic area have antibodies against desmoglein 1. METHODS: We used an enzyme-linked immunosorbent assay to detect antibodies against desmoglein 1 in serum samples from 60 patients with endemic pemphigus foliaceus (fogo selvagem) who lived in Limão Verde or elsewhere in Brazil, 372 normal subjects (without pemphigus foliaceus) from Limão Verde and surrounding locations, and 126 normal subjects from the United States and Japan. RESULTS: Antibodies against desmoglein 1 were detected in 59 of the 60 patients with fogo selvagem (98 percent) but in only 3 of the 126 normal subjects from the United States and Japan (2 percent). Antibodies were also detected in 51 of the 93 normal subjects from Limão Verde (55 percent) and in 54 of the 279 normal subjects from surrounding areas (19 percent). Serum samples obtained one to four years before the onset of disease were available for five patients; all five had antibodies in the initial serum samples, and the onset of disease was associated with a marked increase in antibody values. CONCLUSIONS: The prevalence of antibodies against desmoglein 1 is high among normal subjects living in an area among where fogo selvagem is endemic, and the onset of the disease is preceded by a sustained antibody response. These findings support the concept that the production of antibodies against desmoglein 1 is initiated by exposure to an unknown environmental agent.
Assuntos
Autoanticorpos/sangue , Caderinas/imunologia , Doenças Endêmicas , Pênfigo/imunologia , Autoantígenos/imunologia , Brasil/epidemiologia , Desmogleína 1 , Ensaio de Imunoadsorção Enzimática , Feminino , Humanos , Indígenas Sul-Americanos , Masculino , Pênfigo/sangue , Pênfigo/epidemiologia , Prevalência , Valores de ReferênciaAssuntos
Pênfigo/classificação , Acantólise/imunologia , Alelos , Autoanticorpos/análise , Doenças Autoimunes/classificação , Doenças Autoimunes/patologia , Caderinas/imunologia , Desmogleína 1 , Doenças Endêmicas , Antígenos HLA/genética , Humanos , Hiperpigmentação/patologia , Pênfigo/imunologia , Pênfigo/patologia , Pele/imunologia , Pele/ultraestrutura , Linfócitos T/imunologiaRESUMO
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.
Assuntos
Dípteros , Indígenas Sul-Americanos , Mordeduras e Picadas de Insetos , Pênfigo/epidemiologia , Animais , Brasil/epidemiologia , Bovinos , Cães , Equidae , Feminino , Geografia , Cavalos , Humanos , PrevalênciaRESUMO
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.
Assuntos
Doenças Autoimunes/epidemiologia , Indígenas Sul-Americanos , Pênfigo/epidemiologia , Adolescente , Adulto , Idoso , Autoanticorpos/análise , Doenças Autoimunes/genética , Doenças Autoimunes/imunologia , Brasil , Criança , Análise por Conglomerados , Epiderme/imunologia , Feminino , Humanos , Indígenas Sul-Americanos/genética , Masculino , Pessoa de Meia-Idade , Linhagem , Pênfigo/genética , Pênfigo/imunologia , Prevalência , Fatores de TempoRESUMO
A study on the resident population of 150 inhabitants of Boa Sorte in the Municipality of Corguinho, Mato Grosso do Sul State, Brazil was made, from March 1991 to March 1994, to establish the prevalence of South American Cutaneous Leishmaniasis (SACL), and to characterize the affected population, in an area of recent transmission. Twelve of the inhabitants showed lesions suspected to be SACL, and in 8 cases it was possible to confirm this by biopsy and parasitology. The mucosal form was found in one patient only, the rest showed the following cutaneous forms: ulcerated (3), ulcero-verrucose (1), hyperkeratotic ulcer (1), infiltrated maccule (1), nodule with florid regional adenopathy (1). All patients reacted favorably to treatment with glucantime, with lesion scarring. Side-effects were rare. The parasite isolated from all patients was identified as Leishmania (Viannia) braziliensis. The Montenegro skin test, applied to the 150 inhabitants, showed 32 reactive ones. Of these, six were carriers of the disease, 21 showed sequelae suggestive of the disease and five showed no signs of infection. The age grouping of the cohort ranged from 22 to 78 years, 75% being male. To date, transmission is suspected to be in the peridomicile.