Kikuchi-Fujimoto disease: two cases

Kikuchi-Fujimoto disease, or necrotizing histiocyte lymphadenitis, is a rare anatomo-clinical entity of unknown cause. The patients present painful cervical lymph nodes, fever, and a flu-like syndrome, but the spontaneous course is usually favorable in six months. We observed two cases within six months in our unit. Both patients improved rapidly after diagnostic adenectomy.     

Kikuchi-Fujimoto disease: a case report

We describe a rare but typical case of Kikuchi-Fujimoto disease (KFD). Two subcutaneous nodules appeared suddenly on the right of the neck of a 15-year-old girl. Microscopy of a surgical specimen of the larger nodule showed necrotizing lymphadenitis. Prompt treatment with mepirizole resulted in the disappearance of the smaller nodule. No recurrence was noted during 6 months of follow-up. KFD is a rare, self-limiting, necrotizing histiocytic lymphadenitis that needs to be differentiated from malignant lymphoma. Any nodal biopsy showing fragmented nuclei, necrosis, and karyorrhexis without prominent neutrophils should alert the physician to consideration of KFD, especially in a young woman presenting with cervical lymphadenopathy.     

Kikuchi-Fujimoto disease: a case report

Kikuchi-Fujimoto disease is a rare benign cervical lymphadenopathy, which often affects young adult women. Its etiology and pathogenesis are unknown. We present the case of Kikuchi-Fujimoto disease in the Polish population and analyse the difficulties in differentiating this disease from the systemic lupus erythematosus.     

Fatal evolution of systemic lupus erythematosus associated with Crohn's disease

The authors describe the case of a young Brazilian woman who was treated of ileocolonic Crohn's disease sparing rectum, as confirmed by colonoscopy and histopathological examination. After a 4-year course of sulfasalazine treatment, she presented with skin facial lesions in vespertilio, fever, arthralgias and high titers of anti-ANA and LE cells. A sulfasalazine-induced lupus syndrome was diagnosed, because after sulfasalazine withdrawal and a short course of prednisone, the clinical symptoms disappeared and the laboratory tests returned to normal. Mesalazine 3 g/day was started and the patient remained well for the next 3 years, when she was again admitted with fever, weakness, arthralgias, diplopy, strabismus and hypoaesthesia in both hands and feet, microhematuria, haematic casts, hypocomplementemia and high titers of autoimmune antibodies. A diagnosis of associated systemic lupus erythematosus was made. Although a pulsotherapy with methylprednisolone was started, no improvement was noticed. A cyclophosphamide trial was tried and again no positive results occurred. The patient evolved to severe clinical manifestations of general vasculitis affecting the central and peripheral nervous system and lungs, having a fatal evolution after 2 weeks. Although uncommon, the association of both disease may occur, and the authors call attention to this possibility, making a brief review of literature.     

Kikuchi-Fujimoto disease associated with mixed connective tissue disease

Kikuchi-Fujimoto disease is characterized by painful cervical lymphadenopathy and constitutional symptoms. Microscopical study of lymph nodes shows focal areas of non-suppurative necrosis with histiocytic and plasmacytoid cell infiltrates. The course is usually benign. Often primitive, necrotising histiocytic lymphadenopathy may be associated with autoimmune disorders. We describe the case of a 30-year-old female patient with two 15-day courses of Kikuchi-Fujimoto disease flares within a period of 3 months, occurring in association with mixed connective tissue disease.     

An unusual cause of cervical lymphadenitis: Kikuchi-Fujimoto disease

Cervical lymphadenitis may be the result of diverse conditions in a patient. Clinical and epidemiologic information about cervical lymphadenitis can often lead to a presumptive diagnosis and, thus, limit the number of studies required as well as direct the type of initial therapy administered. We report a case of cervical lymphadenitis in a Vietnamese woman for whom a presumptive diagnosis of tuberculosis was made and antituberculous therapy was started. Pathologic examination of an excised lymph node revealed the correct diagnosis--histiocytic necrotizing lymphadenitis, or Kikuchi-Fujimoto disease. We review the clinical, epidemiologic, and pathologic features of this recently recognized disease. Careful examination of excised material from the lymph nodes should prevent patients who have Kikuchi-Fujimoto disease from receiving unnecessary treatment.     

Catastrophic antiphospholipid syndrome and Kikuchi-Fujimoto disease: the first case reported

The case of a man with diagnosis of Kikuchi-Fujimoto disease (KFD) and catastrophic antiphospholipid syndrome (CAPS) is reported. He presented prolonged fever, lymphadenopathies, arthralgia, weight loss, hepatosplenomegaly and positive IgM for cytomegalovirus. While he was empirically treated with tuberculostatic drugs, he suddenly developed systemic inflammatory response syndrome, multiple organ failure and distal necrosis. On suspicion of severe sepsis, antibiotics, corticoids and recombinant human activated protein C (XIGRIS) were administrated. Exhaustive laboratory searching was negative. Histopathologic examinations of lymph node first disclosed malignant lymphoma but later KFD was confirmed. One month later, laboratory tests showed the presence of antiphospholipid antibodies (aPL). The patient was discharged after two months of hospitalization. This case exhibits a KFD complicated by definite CAPS. Cytomegalovirus could be involved in the development of both CAPS and KFD. Because of the severity of the case, we believe that XIGRIS noticeable improved the altered coagulation profile associated with CAPS.     

Kikuchi-Fujimoto disease: an unusual association with acute renal failure

Kikuchi-Fujimoto disease, also known as histiocytic necrotizing lymphadenitis of unknown etiopathogenesis, is a self-limited disease which frequently appears as feverish lymphadenomegaly, thus creating the need for differential diagnosis with lymphoma, systemic lupus erythematosus (SLE), infectious mononucleosis, cat-scratch disease, and toxoplasmosis with lymphonodal impairment. However, there are cases in which it may evolve with complications such as aseptic meningitis, cerebellar ataxia, and aseptic myocarditis. We are presenting a case of a 24-year-old man who had an initial picture of arthralgia, evening fever and adenomegaly. Kikuchi disease was diagnosed through lymph node biopsy with immunohistochemistry and evolves with severe systemic manifestations, such as pericarditis with cardiac tamponade, pneumonitis, hepatitis, and acute kidney failure - the latter has not been reported in literature yet. There was significant improvement of the clinical picture with prednisone.     

Fatal rhabdomyolysis in systemic lupus erythematosus

The authors describe herein the sixth lupus case that evolved with rhabdomyolysis. A 36-year-old woman with systemic lupus erythematosus was admitted to our hospital with malaise, myalgia, dysphagia, fever, preserved muscle strength, leukocytosis (15,600 cells), and increased creatine kinase of 1,358 IU/L that reached 75,000 IU/L in few days. She denied the use of myotoxic drugs and alcohol. Urine 1 showed false positive for hemoglobinuria (myoglobin) without erythrocytes in the sediment, confirming the diagnosis of rhabdomyolysis. Secondary causes were excluded. She was treated with hyperhydration and alkalinization of urine. Despite treatment, the patient developed pulmonary congestion and she died. The authors also review in this article rhabdomyolysis in patients with systemic lupus erythematosus.     

Arthritis as an unusual manifestation of Kikuchi-Fujimoto disease

SIR, The cases are described of two young women presenting witharthritis, who were diagnosed with Kikuchi-Fujimoto disease(KFD; also called histiocytic necrotizing lymphadenitis). Arthritis has been described previously in patients in whomKFD is associated with other autoimmune disease, such as adultStill's disease and systemic lupus erythematosus (SLE). In ourpatients there was no evidence of coexistent autoimmune disease.To our knowledge, this is the first report of arthritis as apresentation of isolated KFD. We discuss the typical clinicalfeatures of KFD, its aetiology, associations and treatment. Case 1 (NM) was a 36-yr-old woman who recently immigrated toAustralia from Sri Lanka. She had undergone microdiscectomyfor left sciatic pain in Sri Lanka 6 months previously, andtook occasional paracetamol for residual pain. She was otherwisewell. NM was seen by her local doctor for worsening back pain.She displayed no     

Fatal infections in systemic lupus erythematosus: the role of opportunistic organisms

The causes of death were examined in patients with systemic lupus erythematosus (SLE) who were cared for at the University of California, San Francisco and who died after 1969. Of the 44 deaths analyzed, 33 patients had autopsies. Infections were common and often determined to be the cause of death. Overall, infections were present in 55 percent (22/44), and judged to be a cause of death in 30 percent (13/44) of all deaths. The infections could be divided into 2 groups: those due to common bacterial organisms and those due to opportunistic infections. These two types of infections occurred with similar frequency. When compared to common bacterial infections, however, the opportunistic infections were more likely to be first diagnosed at autopsy (p = .001). In only 3 of the 15 patients with an opportunistic infection was the diagnosis made antemortem. Failure to diagnose an opportunistic infection early occurred when the infection simulated active SLE, and when the possibility of an opportunistic infection was not aggressively investigated. The most common opportunistic infections were Candida albicans and Pneumocystis carinii. The most common site of opportunistic infection was the lung. Seventeen patients had 27 common bacterial infections, chiefly sepsis from Staphylococcus aureus and aerobic gram-negative organisms. Eight patients had both a common bacterial and an opportunistic infection. Stepwise linear regression analysis showed that death from infection correlated most strongly with prednisone and cytotoxic drug use in the 3 months before final admission. No measure of lupus activity was found to correlate with death from infection, except that hypocomplementemia correlated with death from bacterial infections.(ABSTRACT TRUNCATED AT 250 WORDS)     

Fatal acute pneumonopathies in disseminated lupus erythematosus

Two cases of systemic lupus erythematosus (SLE) complicated by pneumonia which resulted in death are reported. The first patient, a 21-year old woman, died of acute diffuse lupus pneumonia; the initial and unusual radiological image of "multiple balloons" progressed within 2 months to terminal interstitial fibrosis. The second patient, a 60-year old woman, died of infection on an interstitial pneumonia which turned into severe fibrosis within 16 months. Acute or chronic lupus pneumonia is uncommon, but it may follow a very serious course. Clinically, true lupus pneumonia must be distinguished from all other types of lung involvement in SLE, such as infection, pulmonary haemorrhage or oedema, iatrogenic pathology, thromboembolic disease, etc. The pathogenetic mechanism of pulmonary lesions directly related to SLE is obscure, although some lung biopsy specimens have shown positive immunofluorescence. Concerning treatment, the initial response to corticosteroid therapy is usually very good, especially in the acute forms of the disease. However, in severe cases immunosuppressive drugs or even plasma exchanges must be added to steroids. For treatment to be rapidly initiated the diagnostic procedures must be completed in the early stages of the disease, involving, when necessary, surgical lung biopsy.