BACKGROUND Kikuchi-Fujimoto disease (KFD) is a self-limiting condition that typically presents with lymphadenopathy and fever. It is autoimmune in nature and possibly associated with systemic lupus erythematosus (SLE). The case report highlights an unusual case of KFD associated with autoimmune hemolytic anemia and provided a review for other atypical presentations. Kikuchi Disease, also known as Kikuchi-Fujimoto disease (KFD), is a benign, self-limiting condition of undetermined etiology, that commonly presents with lymphadenopathy and fever. The disease was initially described in young women from Japan. However, it became more apparent that although Kikuchi is frequently reported in Asia, it does occur in various racial groups. In a retrospective literature review by Kucukardali et al, 1 cases were reported from the USA, Taiwan, and Spain, with a male to female ratio of 1:3. Although the pathogenesis is not completely understood, the underlying mechanism is thought to be an immune response to an infectious agent, with some studies reporting Epstein-Barr virus (EBV), 2 Human herpesvirus 6 (HHV6), 3 parvovirus, 4 and others reporting bacterial and parasitic infection. The immune response predominantly involves T cells and histiocytes, in addition to elevated levels of cytokines, markedly interferon-gamma and IL-6. 5 The most common presentation in the majority of cases of KFD is cervical lymphadenopathy. In a literature review by Kucukardali et al, 1 out of 244 cases of KFD, 100% had cervical lymphadenopathy, and 35% of cases complained of fever. Other reported signs and symptoms include rash, arthritis, and hepatosplenomegaly.

INTRODUCTION:Nocardiosis is an opportunistic infection that most commonly manifests as pulmonary disease. Disseminated nocardiosis is rare and is associated with high mortality. We present a case of a woman with disseminated nocardiosis presenting with acute liver failure complicated by intraparenchymal hemorrhage. CASE PRESENTATION:A 30-year-old woman with a history of alcohol abuse presented with jaundice, bloating, and diarrhea for 2 weeks. Laboratory examination revealed hemoglobin of 5.2 g/dL, platelets 60,000/mL, INR 3.4, AST 113 U/L, ALT 38 U/L, and total bilirubin of 30 mg/dL. MRCP revealed a normal biliary tree, nodular liver contour and evidence of portal hypertension. Acetaminophen level and acute viral hepatitis panel were negative. HIV serology and autoimmune hepatitis panel were negative. Chest CT revealed a 5 x 3.5 cm subpleural mass in the left upper lobe with areas of gas density (Fig 1). Her hospital course was complicated by a rapid deterioration in mental status and DIC. Head CT revealed an acute left occipital-parietal intraparenchymal hemorrhage. She was intubated and transferred to the ICU. Bronchoscopy with BAL grew Nocardia otitidiscaviarum. Brain MRI revealed FLAIR signal hyperintensity lining the ventricles, concerning for ventriculitis or abscess (Fig 2). An external ventricular drain was placed for interval development of hydrocephalus. The patient received IV trimethoprim-sulfamethoxazole and intrathecal amikacin. Given her poor prognosis, her family elected to withdraw care. DISCUSSION: Nocardia is a branching, filamentous gram-positive bacilli found ubiquitously in soil. Infection usually occurs by inhalation of aerosolized bacteria resulting in predominant lung involvement with potential hematogenous spread to other organs. Patients with impaired T-cell mediated immunity, such as HIV, chronic steroid use, diabetes, or alcoholism are at risk of developing severe infection. Our patient had a longstanding history of heavy alcohol use, likely predisposing her to disseminated infection. Nocardiosis is considered disseminated when two or more nocardial abscesses are found at two or more locations. Up to 20% of all Nocardia infections involve the brain. Current guidelines recommend imaging of the brain for any patient with confirmed or suspected pulmonary nocardiosis. Prognosis is usually poor, and mortality can be as high as 64%. First-line treatment is trimethoprim-sulfamethoxazole. However some species, such as N. otitidiscaviarum found in our patient, are occasionally resistant. Due to suspected CNS involvement, dual coverage was recommended with the addition of IV amikacin at dosing for CNS penetrance. CONCLUSIONS:In patients presenting with a cavitary lung lesion and associated brain lesion, nocardiosis should be considered in the differential. Early brain imaging may be warranted in patients presenting with severe disease.

Kikuchi-Fujimoto Disease (KFD) is a self-limiting condition that typically presents with lymphadenopathy and fever. It's autoimmune in nature, and possibly associated with systemic lupus erythematosus (SLE). The case report highlights an unusual case of KFD associated with autoimmune hemolytic anemia and provided a review for other atypical presentations.