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Doença de Kikuchi-Fujimoto : caso clínico na Pediatria
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A doença de Kikuchi-Fujimoto ou linfadenite histiocítica necrosante é uma patologia de etiologia desconhecida, de muito baixa prevalência, afetando principalmente mulheres jovens asiáticas. É geralmente autolimitada e apresenta-se como linfadenite cervical e febre. Pode também causar exantema e artrite. Sendo uma doença rara e afetando com maior incidência uma população específica, muitas vezes esta patologia não é equacionada no diagnóstico diferencial de linfadenite ou febre sem foco. O objetivo deste trabalho é descrever um caso clínico desta patologia e fazer uma breve revisão sobre o tema. Apresenta-se o caso de uma criança, do sexo masculino, com 7 anos, previamente saudável, internado por febre prolongada com 13 dias de evolução, astenia, anorexia, emagrecimento, exantema, odinofagia e artralgias. No exame objetivo detetou-se adenomegália cervical, hepatoesplenomegália, exantema salmão evanescente no tronco e membros e poliartrite. O ecocardiograma era normal. Das análises realizadas destaca-se leucocitose e elevação dos parâmetros inflamatórios (39 980 leucócitos/uL; Neutrófilos 80%; Linfócitos 13%: Proteína C reativa 18,3 mg/dL: VS 92mm/h; ferritina 11 122ng/mL). Excluiu-se infeção por Streptococcus pyogenes, Bartonella henselae, Mycobacterium tuberculosis, toxoplasmose, vírus da imunodeficiência humana, Epstein-Barr, citomegalovírus e herpes. Os anticorpos antinucleares eram negativos e o estudo do complemento normal. O esfregaço de sangue periférico era normal. Realizou biópsia osteomedular, excluindo doença linfoproliferativa. Realizou biópsia do gânglio cervical, tendo-se detetado linfadenite histiocítica necrosante e histiocítica, compatível com doença de Kikuchi-Fujimoto. Iniciou tratamento com prednisolona (1mg/Kg/dia) com rápida resposta, realizando diminuição progressiva da dose. Após 4 meses de seguimento em consulta de Reumatologia Pediátrica encontra-se assintomático e sem terapêutica.
Kikuchi-Fujimoto disease or histiocytic necrotizing lymphadenitis is a rare disease of unknown aetiology, which affects mainly young Asian women. It is usually self-limited and presents as cervical lymphadenitis and fever. It can also cause rash and arthritis. Due to its rarity, this disease is not often considered in the differential diagnosis of patients with lymphadenitis or fever of unknown origin. The main goal of this work is to describe a child with Kikuchi-Fujimoto disease and review characteristics of this disease. We present a 7 year-old male, previously healthy, admitted to the hospital for a 13-day fever of unknown origin, fatigue, anorexia, weight loss, odynophagia, rash and arthralgias. In the physical examination, it was found a cervical lymphadenopathy, hepatosplenomegaly, salmon evanescent rash in the torso and limbs and polyarthritis. The echocardiogram was normal. The laboratory tests showed leucocytosis and elevation of the inflammation parameters (39 980 leucocytes/uL; neutrophils 80%, lymphocytes 13%; C-reactive protein 18.3 mg/dL; ESR 92mm/h; ferritin 11 122ng/mL). It was excluded infections due to Streptococcus pyogenes, Bartonella henselae, Mycobacterium tuberculosis, human immunodeficiency virus, Epstein-Barr virus, cytomegalovirus and herpes simplex virus. The anti-nuclear antibodies were negative, and C3 and C4 were normal. The peripheral blood smear was normal. The osteomedullary biopsy excluded lymphoproliferative disorders. The cervical lymph node biopsy showed histiocytic necrotizing lymphadenitis, compatible with Kikuchi-Fujimoto disease. The patient was treated with prednisolone (1mg/Kg/day). There was a fast response to treatment. Steroids were progressively reduced and stopped. After four months the patient is asymptomatic and without medication.
Kikuchi-Fujimoto disease or histiocytic necrotizing lymphadenitis is a rare disease of unknown aetiology, which affects mainly young Asian women. It is usually self-limited and presents as cervical lymphadenitis and fever. It can also cause rash and arthritis. Due to its rarity, this disease is not often considered in the differential diagnosis of patients with lymphadenitis or fever of unknown origin. The main goal of this work is to describe a child with Kikuchi-Fujimoto disease and review characteristics of this disease. We present a 7 year-old male, previously healthy, admitted to the hospital for a 13-day fever of unknown origin, fatigue, anorexia, weight loss, odynophagia, rash and arthralgias. In the physical examination, it was found a cervical lymphadenopathy, hepatosplenomegaly, salmon evanescent rash in the torso and limbs and polyarthritis. The echocardiogram was normal. The laboratory tests showed leucocytosis and elevation of the inflammation parameters (39 980 leucocytes/uL; neutrophils 80%, lymphocytes 13%; C-reactive protein 18.3 mg/dL; ESR 92mm/h; ferritin 11 122ng/mL). It was excluded infections due to Streptococcus pyogenes, Bartonella henselae, Mycobacterium tuberculosis, human immunodeficiency virus, Epstein-Barr virus, cytomegalovirus and herpes simplex virus. The anti-nuclear antibodies were negative, and C3 and C4 were normal. The peripheral blood smear was normal. The osteomedullary biopsy excluded lymphoproliferative disorders. The cervical lymph node biopsy showed histiocytic necrotizing lymphadenitis, compatible with Kikuchi-Fujimoto disease. The patient was treated with prednisolone (1mg/Kg/day). There was a fast response to treatment. Steroids were progressively reduced and stopped. After four months the patient is asymptomatic and without medication.
Descrição
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2021
Palavras-chave
Doença de Kikuchi-Fujimoto Linfadenite Artrite Exantema Pediatria