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An Atypical Presentation of Kikuchi-Fujimoto Disease Mimicking Systemic Lupus Erythematosus: Case Report and Literature Review

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An Atypical Presentation of Kikuchi-Fujimoto Disease Mimicking Systemic Lupus Erythematosus: Case Report and Literature Review Open Journal of Pediatrics, 2011, 1, 67-71 OJPed doi:10.4236/ojped.2011.14016 Published Online December 2011 (http://www.SciRP.org/journal/ojped/) An atypical presentation of Kikuchi-Fujimoto disease mimicking systemic lupus erythematosus: case report and literature review Diane Belder-Preston1*, Catherine-Maude Pound1,2, Roman Jurencak1,3 1Department of Pediatrics, Children’s Hospital of Eastern Ontario, Ottawa, Canada; 2Division of Pediatric Medicine, Children’s Hospital of Eastern Ontario, Ottawa, Canada; 3Division of Rheumatology, Children’s Hospital of Eastern Ontario, Ottawa, Canada. Email: *[email protected] Received 1 October 2011; revised 5 November 2011; accepted 18 November 2011. ABSTRACT 1. INTRODUCTION Purpose: To report a case of atypical Kikuchi-Fuji- Kikuchi disease, also called Kikuchi-Fujimoto Disease moto disease (KFD) that illustrates several overlap- (KFD) and Histiocytic Necrotizing Lymphadenopathy, is ping features with systemic lupus erythematosus a rare, benign, self-limited disease of unknown etiology. (SLE). Methods: A case is reported followed by a re- KFD was first reported in 1972 by Kikuchi [1] as well as view of the current literature. Case Report: A 16-year- independently, by Fujimoto et al. [2]. Typical features of old boy with an unusual manifestation of Kikuchi- the disease include subacute regional lymphadenopathy Fujimoto disease (KFD) is described. The patient predominantly involving cervical lymph nodes as well as presented with fever, weight loss and severe abdomi- fevers, often accompanied by leukopenia, high erythro- nal pain, due to extensive necrotizing retroperitoneal cyte sedimentation rate (ESR), and anemia [3]. Exci- and mesenteric lymphadenopathy. During the course sional biopsy of the affected lymph node shows non- specific histopathologic features of cortical and paracor- of his illness, he developed several symptoms sugges- tical necrosis. No specific diagnostic imaging or labora- tive of systemic lupus erythematosus (SLE): a peri- tory tests are available and KFD remains a diagnosis of cardial effusion, cotton wool spots on the retina and exclusion. Clinicians must rule out other causes of ne- antibodies against nuclear antigens (ANA), Smith crotizing lymphadenopathy, such as malignancy, sys- (Sm) and ribonucleoprotein (RNP) antigens. However, temic connective tissue disorders, and infectious lym- no additional features of SLE were found. The pa- phadenitis, before diagnosing KFD. These other diagno- tient subsequently fully recovered within two months, ses have different therapeutic and prognostic implica- without initiation of immunosuppressive therapy. His tions. This can result in costly and invasive investiga- autoantibodies became negative five months after tions, potentially causing significant distress to the pa- initial presentation and he remains well at his 23 tient and family. month follow up visit. Discussion: We hypothesize We describe the case of a young man who presented that the autoantibodies developed by our patient to a pediatric tertiary care centre with atypical features were secondary to self-antigen induced autoimmunity of Kikuchi-Fujimoto Disease. related to his extensive tissue necrosis. Despite ini- tially having clinical features suggestive of SLE, our 2. CASE REPORT patient’s full and spontaneous recovery strongly A 16-year-old, previously healthy, Caucasian boy pre- supports the diagnosis of KFD. This illustrates the sented to a pediatric tertiary care centre with a three- need for careful diagnosis, in order to avoid unneces- week history of intermittent abdominal pain, vomiting, sary and potentially toxic treatment with immuno- anorexia and a seven-kilogram weight loss. He also had suppressive agents. a two-week history of intermittent fevers, up to 40 de- grees Celsius, associated with a faint diffuse erythema- Keywords: Kikuchi-Fujimoto’s Disease; Retroperitoneal tous rash involving the face and upper torso. Personal Lymphadenitis; Systemic Lupus Erythematosus (SLE); and family histories were noncontributory. Antinuclear Antibodies (ANA) Physical examination on admission revealed a pale, Published Online December 2011 in SciRes. http://www.scirp.org/journal/OJPed 68 D. Belder-Preston et al. / Open Journal of Pediatrics 1 (2011) 67-71 thin boy, weighing 59.7 kg. His vital signs were: heart tiple retinal cotton wool spots. Cardiac echocardiogra- rate 120, blood pressure 121/81, respiratory rate 16 and phy demonstrated a moderate-sized pericardial effusion oxygen saturation 98% in room air. He was afebrile with with no hemodynamic compromise. Magnetic Reso- a faint malar flush. His abdomen was non-acute but dif- nance Angiography of the abdomen, as well as a Mag- fusely tender with no hepatosplenomegaly. The remain- netic Resonance Imaging of the hip and shoulder girdles der of the examination was unremarkable. No peripheral helped to exclude the diagnoses of vasculitis and myosi- lymphadenopathy was appreciated. tis. Laboratory investigations showed elevated inflamma- During his hospitalization, our patient received mainly tory markers, mild non-hemolytic anemia, transaminitis supportive care after a brief three-day course of a third and markedly elevated lactate dehydrogenase (LDH) generation cephalosporin antibiotic pending negative (Table 1). Autoantibodies (anti-nuclear antibodies (ANA), cultures. The severity of his abdominal pain was such anti-neutrophil cytoplasmic antibodies, rheumatoid fac- that he required morphine, gabapentin, ketorolac and tor and antiphospholipid antibodies) were all negative. clonidine. Because of poor appetite, food intolerance and Complement (C3, C4) and total immunoglobulin levels ongoing weight loss, total parenteral nutrition was initi- (IgG, IgA and IgM) were within normal limits. ated. A Computerized Tomography scan of his abdomen Atypical Kikuchi-Fujimoto disease was suspected demonstrated significant lymphadenopathy in both the based on the presence of necrotizing non-granulomatous retroperitoneum and the root of the mesentery. Explor- retroperitoneal lymphadenitis and the exclusion of other ative laporotomy with lymph node biopsy showed ne- disorders. Intravenous immunoglobulin therapy was crotizing lymphadenitis with extensive tissue necrosis, offered but declined by the patient’s family. Steroid without evidence of neutrophilic inflammation or pres- therapy was not offered as there was still a degree of ence of granulomas. In one section, a vessel with intra- uncertainty in terms of the diagnosis of KFD at that mural chronic inflammation suspicious for vasculitis was stage. observed. Special stains for infectious organisms and With supportive management only, the patient’s clini- immunophenotyping for neoplastic cells yielded nega- cal status improved. By day 30 of hospitalization, he was tive results. ambulating, tolerating increasing naso-gastric feeds and Given the absence of a definitive diagnosis, further demonstrating consistent weight gain. At the family’s investigations were performed. These included bone request, the patient was transferred to another tertiary marrow studies to rule out malignancy, upper and lower care centre for a second opinion. endoscopies with biopsies to rule out atypical inflam- At the second hospital, approximately one month after matory bowel disease and Whipple’s disease, as well as his initial biopsy, the patient underwent another laporo- an extensive infectious work up. Tuberculosis, Epstein- tomy with lymph node biopsy. Extensive bland necrosis Barr virus, toxoplasmosis, parvovirus, cytomegalovirus, with numerous macrophages was reported. Histiocytes, Streptococcus pneumonia, and fungal infections were lymphocytes and minor plasma cell populations were excluded. Ophthalmologic examination uncovered mul- scattered throughout. Again, no neutrophilic infiltration Table 1. Laboratory data. Reference range During admission Eight weeks after admission Five months after admission ESR (mm/Hr) 0 - 10 63 13 6 CRP (mg/L) <8.0 15 <1 <1 Ferritin (ug/L) 24 - 336 1408 968 WBC (×109 L) 4.5 - 11 10.6 4.85 4.5 Hb (g/L) 120 - 160 90 129 153 Plt (×109 L) 150 - 450 382 393 229 AST (U/L) 14 - 50 265 19 26 ALT (U/L) 10 - 55 143 17 20 LDH (U/L) 300 - 700 3016 399 468 ESR, erythrocyte sedimentation rate; CRP, C reactive protein; WBC, White blood cell; Hb, Hemoglobin; Plt, Platelet; AST, Aspartate aminotransferase; ALT, Alanine transaminase; LDH, lactate dehydrogenase. Copyright © 2011 SciRes. OJPed D. Belder-Preston et al. / Open Journal of Pediatrics 1 (2011) 67-71 69 or granuloma was seen. Absence of hematoxylin bodies prehensive review reported a ratio closer to 1:1 [11]. In was noted. No infectious etiology was identified. On the pediatric population, the ratio seems to be higher in repeat testing, some of the patient’s autoantibodies and boys with reported ratios ranging from 1.4 to 2.8:1 [5, immunoserologies became positive (Table 2). Labora- 6]. tory methods used to detect these autoantibodies were KFD typically presents with cervical lymphadenopa- identical at both hospitals (ANA by indirect immuno- thy. Low-grade fevers, fatigue, skin rashes affecting the fluorescence using Hep-2 cell line as substrate, ENA by face and the upper body, nausea, vomiting, diarrhea and ELISA). Nonetheless, he continued to improve with weight loss are also reported [7,9,11]. Cases of non-cer- supportive treatment only, and no immunosuppressive vical lymphadenopathy have been described; generalized, therapy was initiated. There was full resolution of his axillary, iliac and retroperitoneal
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