Overlap between Systemic Lupus Erythematosus and Kikuchi Fujimoto Disease

Abstract

Case presentationA 33-year-old African-Caribbean man with a history of possible Kikuchi Fujimoto disease (KFD) versus systemic lupus erythematosus (SLE) was evaluated for 3 weeks of fevers to 38.9°C and cough accompanied by fatigue, anorexia, weight loss, diffuse rash, lymphadenopathy, and oral and nasal ulcers.His past medical history was significant for a hospitalization for febrile neutropenia 2 years prior to this evaluation.At that time, he presented with 2 weeks of fever, shaking chills, malaise, arthralgia, and myalgia.This had been preceded by 2 months of fatigue, 10-lb weight loss, and the development of scarring alopecia.He was noted at this time to have had diffuse lymphadenopathy, including the occipital, anterior, and posterior cervical regions.He also was found to have malar erythema, oral ulcers, and a rash on his scalp, ears, face, chest, arms, and legs.The rash was characterized by erythematous, hyperpigmented papules, and plaques with scale, follicular plugging, and areas of scarring.He was treated empirically with intravenous (i.v.) piperacillin/tazobactam as his work-up ensued.His treating physicians were specifically concerned for an underlying malignancy, an autoimmune process, or an infectious process.His laboratory tests from his initial presentation showed anemia, leucopenia, an elevated erythrocyte sedimentation rate, and a twice normal level of lactate dehydrogenase (LDH; Table 1).Posteroanterior and lateral radiographs of the chest were normal.Blood cultures, urine cultures, and serologic work-up for a viral infection were negative (Table 2).Positive serologies for autoimmune disease included an anti-nuclear antibody (ANA) in a speckled pattern, Smith, ribonucleoprotein (RNP), and Ro, and a low C3 level (Table 2).Biopsies of the skin and one cervical lymph node were consistent with KFD.The patient was discharged from the hospital and followed by his internist with the putative diagnosis of KFD versus SLE.The internist recommended rheumatologic consultation, but the patient was not able to follow through.The patient was treated with hydroxychloroquine 200 mg daily as prescribed by his internist and felt generally well up until the time of his second hospitalization.He reported that if he missed his hydroxychloroquine for a few days, he would develop arthralgias and malaise.At the time of his second admission, 2 years later, the patient complained of a mildly productive cough with dyspnea, fevers, malaise, increased rash, and increased lymphadenopathy.He described a preceding upper respira-