Publicação
Doença de Kikuchi-Fujimoto : a propósito de um caso clínico na pediatria
| Resumo: | Kikuchi-Fujimoto’s Disease or Histiocytic Necrotizing Lymphadenitis (KFD/HNL) is a benign, self-limited, rare disease of unknown etiology, which is characterized by cervical lymphadenopathies associated to a febrile syndrome, simulating various clinical entities that possess different evolution and therapeutic choices. Subcutaneous Panniculitis T-Cell Lymphoma (SPTCL) is considered a peripheral lymphoma, derived from cytotoxic maturation that mimics a panniculitis process. It can clinically present subcutaneous nodules or plaques, without superficial lesions, erosive or necrotic lesions. A definitive diagnostic is given by an anatomic-pathological evaluation of a ganglionic biopsy. Rarer is the association of these two diseases with the Hemophagocytic Syndrome, also known as Hemophagocytic Limphohistiocytosis (HPS/HLH), an excessive inflammatory reaction syndrome with tissue destruction due to an abnormal immune activation. It will be described a clinical situation in paediatric age, characterized by fever and lymphadenopathies associated to the appearance of oedema and erosive lesions of the labial mucosa. The initial investigation permitted the identification of HPS/HLH, which was associated to SPTCL as underlying disease. Later, it is concluded that it is KFD/HNL. It will be discussed the etiopathogenesis, differential diagnosis, therapy and prognosis in a patient with this presentation. |
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| Autores principais: | Tomás, Tiago Filipe da Cruz |
| Assunto: | Doença de Kikuchi-Fujimoto Pediatria |
| Ano: | 2014 |
| País: | Portugal |
| Tipo de documento: | dissertação de mestrado |
| Tipo de acesso: | acesso restrito |
| Instituição associada: | Universidade de Lisboa |
| Idioma: | português |
| Origem: | Repositório da Universidade de Lisboa |
| Resumo: | Kikuchi-Fujimoto’s Disease or Histiocytic Necrotizing Lymphadenitis (KFD/HNL) is a benign, self-limited, rare disease of unknown etiology, which is characterized by cervical lymphadenopathies associated to a febrile syndrome, simulating various clinical entities that possess different evolution and therapeutic choices. Subcutaneous Panniculitis T-Cell Lymphoma (SPTCL) is considered a peripheral lymphoma, derived from cytotoxic maturation that mimics a panniculitis process. It can clinically present subcutaneous nodules or plaques, without superficial lesions, erosive or necrotic lesions. A definitive diagnostic is given by an anatomic-pathological evaluation of a ganglionic biopsy. Rarer is the association of these two diseases with the Hemophagocytic Syndrome, also known as Hemophagocytic Limphohistiocytosis (HPS/HLH), an excessive inflammatory reaction syndrome with tissue destruction due to an abnormal immune activation. It will be described a clinical situation in paediatric age, characterized by fever and lymphadenopathies associated to the appearance of oedema and erosive lesions of the labial mucosa. The initial investigation permitted the identification of HPS/HLH, which was associated to SPTCL as underlying disease. Later, it is concluded that it is KFD/HNL. It will be discussed the etiopathogenesis, differential diagnosis, therapy and prognosis in a patient with this presentation. |
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