Report of a rare case of histiocytic necrotizing lymphadenitis with bilateral pleural effusion diagnosed via cervical lymph node biopsy

ABSTRACT CONTEXT: Histiocytic necrotizing lymphadenitis (HNL) is a rare disorder that is often benign and self-limiting. There have been reports of co-occurrence of HNL with other diseases, including systemic lupus erythematosus, hemophagocytic syndrome and antiphospholipid syndrome. CASE REPORT: Here, we report a case in which a patient experienced unexplained fever, swelling of the cervical lymph node and bilateral pleural effusion and was ultimately diagnosed with HNL based on results from a lymph node biopsy. After treatment with glucocorticoid, the patient regained normal body temperature, the swelling of the lymph nodes disappeared and the pleural effusion was reabsorbed. CONCLUSIONS: The pathogenesis of HNL remains unclear, and pleural effusion is rarely reported in HNL patients. We presented this case to improve diagnostic awareness of this condition among clinicians and help reduce the likelihood of misdiagnosis.

Kikuchi-Fujimoto disease: three case reports / Doença de Kikuchi-Fujimoto: relato de três casos

CONTEXT: Kikuchi-Fujimoto disease (KFD) manifests in most cases as unilateral cervical lymphadenomegaly, with or without accompanying fever. The disease mainly affects young women and has a self-limited course. It is more common in oriental countries, with few reports of its occurrence in Brazil. KFD should be included in the differential diagnosis of suspected cases of viral infections, tuberculosis, reactive lymphadenitis, systemic lupus erythematosus and metastatic diseases. It can be histologically confused with lymphoma. The disease is benign and self-limiting and an excisional biopsy of an affected lymph node is necessary for diagnosis. There is no specific therapy. CASE REPORTS: This study reports on three cases of non-Asian female patients with KFD who were attended at our service between 2003 and 2006. A review of the literature was carried out, with a systematic search on this topic, with the aim of informing physicians about this entity that is manifested by cervical masses and fever.

CONTEXTO: A doença de Kikuchi-Fujimoto (DKF) se manifesta na maioria das vezes como linfadenomegalia cervical unilateral, que pode ser acompanhada de febre. Afeta principalmente mulheres jovens, com evolução autolimitada. A prevalência desta doença é mais comum no oriente, havendo poucos relatos de sua ocorrência em nosso meio. DKF deve ser incluída no diagnóstico diferencial de casos suspeitos de infecções por vírus, tuberculose, linfadenite reacional, lúpus eritematoso sistêmico e doença metastática. Histologicamente pode ser confundida com linfoma. A doença é de caráter benigno, auto-limitada, a biópsia excisional do linfonodo acometido é necessária para o diagnóstico e não há tratamento específico. RELATO DE CASOS: Este trabalho relata três casos de pacientes não asiáticas do sexo feminino com DKF, atendidas em nosso serviço entre 2003 e 2006, e faz revisão da literatura com uma busca sistematizada sobre o assunto, com o objetivo de informar os médicos sobre essa entidade que se manifesta com massa cervical e febre.

Doença de Kikuchi-Fujimoto / Kikuchi-Fujimoto disease

A doença de Kikuchi-Fujimoto é caracterizada por febre e linfadenopatia, geralmente cervical. Esta doença acomete principalmente mulheres jovens. Pode ser confundida com linfoma, metástase de adenocarcinoma e tuberculose. Relatamos dois casos da doença de Kikuchi-Fujimoto. No primeiro caso, uma paciente de 28 anos havia tratado tuberculose há um ano e apresentava quadro clínico e histológico compatível com a doença de Kikuchi-Fujimoto. A segunda paciente, de 58 anos, recebeu tratamento inicialmente para granulomatose de Wegener e, posteriormente, para tuberculose. O exame histopatológico com estudo imunohistoquímico permitiu estabelecer o diagnóstico da doença de Kikuchi-Fujimoto nos dois casos. Após o diagnóstico definitivo, ambas foram tratadas sintomaticamente e melhoraram clinicamente dentro de um mês. Posteriormente, a segunda paciente desenvolveu lúpus eritematoso sistêmico.

Kikuchi-Fujimoto disease is characterized by fever and lymphadenopathy, usually localized in the cervical region. This disease principally affects young females. It can be confused with lymphoma, adenocarcinoma metastasis and tuberculosis. We report two cases of Kikuchi-Fujimoto disease. In the first case, a 28-year-old female had been treated for tuberculosis one year prior and presented with a clinical and histological profile compatible with Kikuchi-Fujimoto disease. The second patient, a 58-year-old female, initially received treatment for Wegener's granulomatosis and, subsequently, for tuberculosis. Histopathological examination followed by immunohistochemical analysis confirmed the diagnosis of Kikuchi-Fujimoto disease in both cases. After the definitive diagnosis had been made, both patients were treated symptomatically, and both presented clinical improvement within one month. Subsequently, the latter patient developed systemic lupus erythematosus.

Uveíte anterior como manifestação da Doença de Kikuchi e Fujimoto / Anterior Uveitis as an ocular manifestation of Kikuchi and Fujimoto's Disease

Apresentação de um caso de febre de origem obscura numa paciente feminina de 35 anos, com queda do estado geral, adenomegalia cervical posterior, monilíase oral, parotidite e irite.Após o parecer oftalmológico, o tratamento foi iniciado e posteriormente com o resultado do exame histopatológico de um linfonodo, diagnosticou-se a Doença de Kikuchi e Fujimoto. Sugerimos que a uveíte anterior seja reconhecida como mais um sinal de suspeita desta doença. São comentados os achados oculares, os aspectos histopatológicos e o tratamento da Doença de Kikuchi e Fujimoto.

Report of a case on Kikuchi and Fujimoto's Disease in a young lady who developed a long standing spiking fever, weight loss, cervical adenomegalia, oral moniliasis, parotiditis and iritis.The histopathological findings, course and treatment as well as the importance of a multidisciplinar approach are commented.