RESUMO
Langerhans' cell histiocytosis represents a diverse group of rare disorders characterized by proliferation and infiltration of various organs by bone marrow-derived abnormal Langerhans' cells. These diorders exhibit varied patterns of organ involvement, age distribution, prognosis and clinical manifestation. Here in we discuss an adult presentation of the disease in a 43 years old lady and summarize the latest recommendations regarding the classification, pathogenesis, work up and treatment of adult patients with these disorders.
Assuntos
Histiocitose de Células de Langerhans/patologia , Células de Langerhans/patologia , Adulto , Anti-Inflamatórios/uso terapêutico , Antineoplásicos Fitogênicos/uso terapêutico , Biópsia , Etiópia , Feminino , Histiocitose de Células de Langerhans/tratamento farmacológico , Humanos , Imuno-Histoquímica , Prednisolona/uso terapêutico , Resultado do Tratamento , Vimblastina/uso terapêuticoAssuntos
Dermatite Irritante/etiologia , Dermatite Ocupacional/etiologia , Dermatoses da Mão/etiologia , Lobelia/efeitos adversos , Adolescente , Adulto , Dermatite Irritante/diagnóstico , Dermatite Irritante/epidemiologia , Dermatite Ocupacional/diagnóstico , Dermatite Ocupacional/epidemiologia , Surtos de Doenças , Etiópia/epidemiologia , Dermatoses da Mão/diagnóstico , Dermatoses da Mão/epidemiologia , Humanos , Testes do Emplastro , Adulto JovemRESUMO
BACKGROUND: Cutaneous leishmaniasis is a vector-borne disease that is in Ethiopia mainly caused by the parasite Leishmania aethiopica. This neglected tropical disease is common in rural areas and causes serious morbidity. Persistent nonhealing cutaneous leishmaniasis has been associated with poor T cell mediated responses; however, the underlying mechanisms are not well understood. METHODOLOGY/PRINCIPAL FINDINGS: We have recently shown in an experimental model of cutaneous leishmaniasis that arginase-induced L-arginine metabolism suppresses antigen-specific T cell responses at the site of pathology, but not in the periphery. To test whether these results translate to human disease, we recruited patients presenting with localized lesions of cutaneous leishmaniasis and assessed the levels of arginase activity in cells isolated from peripheral blood and from skin biopsies. Arginase activity was similar in peripheral blood mononuclear cells (PBMCs) from patients and healthy controls. In sharp contrast, arginase activity was significantly increased in lesion biopsies of patients with localized cutaneous leishmaniasis as compared with controls. Furthermore, we found that the expression levels of CD3ζ, CD4 and CD8 molecules were considerably lower at the site of pathology as compared to those observed in paired PBMCs. CONCLUSION: Our results suggest that increased arginase in lesions of patients with cutaneous leishmaniasis might play a role in the pathogenesis of the disease by impairing T cell effector functions.