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Query: UMLS:C0409974 (lupus)
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Kikuchi-Fujimoto disease, also known as hystiocytic necrotizing lymphadenitis, is an unusual entity which affects predominantely young Asiatic females, although its distribution is world-wide. Cardinal symptoms are fever and adenopathy, generally cervical, although generalized and extraganglionar cases have been described. Considered a self-limiting disease, it has to be differentiated by pathologic analysis from other less-benign disorders such as lymphoma or histiocytoma, which bear worse prognosis and may require specific treatment. Although the origin of this disease is unknown, a viral origin is postulated and the immune system is involved Kikuchi disease has been associated to other entities such as subcutaneous lupus erythematosus, Hashimoto thyroiditis, etc... requiring that patients be followed immunologically alter diagnosis of this disease. We present a case of Kikuchi disease associated with subacute lymphocytic thyroiditis.
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PMID:[Kikuchi's disease: a case report and literature review]. 1283 Dec 99

We describe an 11-year-old boy who had several, asymptomatic, erythematous papules in the oropharynx and larynx with recent onset, two cervical lymphadenopathies, and a painless, erythematous plaque on the right wrist with a 2.5-year history of slow growth. Histologic examination of the mucocutaneous lesions revealed a submucous infiltrate of lymphocytes and Langhans giant cells in the papules and granulomatous dermatitis in the plaque. The cervical lymph node was biopsied and on the surgical scar, an erythematous, nodular lesion developed. A biopsy specimen of this lesion showed tuberculoid granulomas with prominent caseation necrosis, and culture was positive for Mycobacterium tuberculosis. The Mantoux test was strongly positive with a vesicular response. A diagnosis of mucocutaneous lupus vulgaris and scrofuloderma secondary to cervical tuberculous lymphadenitis was made. Two months after initiation of antituberculosis therapy there was a complete resolution of mucous lesions and healing with atrophic scars on the neck and wrist. This is a rare presentation in the literature and reminds clinicians that tuberculosis should be kept in mind in the differential diagnosis of oral cavity lesions.
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PMID:Tuberculosis in a child presenting as asymptomatic oropharyngeal and laryngeal lesions. 1452 63

A 54-year-old man of Persian origin presented to our department with a 1-year history of ulcers on the right leg that had been unresponsive to numerous topical treatments, accompanied by lymphedema of the right leg. Medical history included hypergonadotropic hypogonadism, which had not been further investigated. He was treated for 20 years with testosterone IM once monthly, which he stopped a year before the current hospitalization for unclear reasons. The patient reported no congenital lymphedema. Physical examination revealed two deep skin ulcers (Figure 1) on the right leg measuring 10 cm in diameter with raised irregular inflammatory borders and a boggy, necrotic base discharging a purulent hemorrhagic exudate. Bilateral leg pitting edema and right lymphangitis with lymphadenitis were noted. He had low head hair implantment, sparse hair on the body and head, hyperpigmentation on both legs, onychodystrophia of the toenails (mainly the large toe and less prominent on the other toes), which was atrophic lichen-planus-like in appearance and needed no trimming (Figure 2), normal hand nails, oral thrush, and angular cheilitis. Other physical findings were gynecomastia, pectus excavatum, small and firm testicles, long extremities, asymmetrical goiter, systolic murmur 2/6 in left sternal border, and slow and inappropriate behavior. The patient's temperature on admission was 39 degrees C. Blood cultures were negative for bacterial growth. Results of laboratory investigations included hemoglobin (11.2 g/dL), hematocrit (26.8%), normal mean corpuscular volume and mean corpuscular hemoglobin volume, and red blood cell distribution width (16%). Blood smear showed spherocytes, slight hypochromia, anisocytosis, macrocytosis, and microcytosis. Blood chemistry values were taken for iron (4 micro g/dL [normal range 40-150 micro g/dL]), transferrin (193 mg/dL [normal range 220-400 mg/dL]), ferritin (1128 ng/mL [normal range 14-160 ng/mL]), transferrin saturation (1.5% [normal range 20%-55%]), serum folate (within normal limits), and vitamin B12 (within normal limits). Direct Coombs' test equaled positive 2 + IgG. All these values indicated anemia of chronic diseases combined with hemolytic anemia. Further blood work-up tested antinuclear antibody (positive <1:80 homogeneous pattern), rheumatoid factors (143 IU/mL [positive >8.5 IU/mL]), C-reactive protein (286 mg/L [normal range 0-5 mg/L]), anticardiolipin IgM antibody (9.0 monophosphoryl lipid U/mL [normal range 0-7.00 MPL U/mL]) and antithrombin III activity (135% [normal range 74%-114%]). Results of other blood tests were within normal limits or negative, including lupus anticoagulant, beta2 glycoprotein, anticardiolipin IgG Ab, anti-ss DNA Ab, C3, C4, anti-RO, anti-LA, anti-SC-70, anti-SM Ab, P-ANCA, C-ANCA, TSH, FT4, anti-T microsomal, antithyroglobulin, protein C activity, protein S free, cryoglobulins, serum immunoelectrophoresis, VDRL, hepatitis C antibodies, hepatitis B antigen, and human immunodeficiency virus. Endocrinological work-up examined luteinizing hormone (22.9 mIU/mL [normal range for adult men 0.8-6 mIU/mL]), follicle stimulating hormone (49.7 mIU/mL [normal range for adult men 1-11 mIU/mL]), testosterone (0.24 ng/mL [normal range for adult men 2.5-8.0 ng/mL]), bioavailable testosterone (0.02 ng/mL [normal range for adult men >0.6 ng/mL]), and percent bioavailable test (8.1% [normal value >20%]). These results indicate hypergonadotropic hypogonadism. Plasminogen activator inhibitor 1 was 6 U (normal value 5-20 U/mL). Karyotyping performed by G-banding technique revealed a 47 XXY karyotype, which is diagnostic of Klinefelter's syndrome. Doppler ultrasound of the leg ulcers disclosed partial thrombus in the distal right femoral vein. X-rays and bone scan displayed osteomyelitis along the right tibia. Histological examination of a 4-mm punch biopsy from the ulcer border revealed hyperkeratosis, acanthosis, hypergranulosis, and mixed inflammatory infiltrate containing eosinophils compatible with chronic ulcer. Multiple vessels were seen, compatible with a healing process. Direct immunofluorescence of the biopsy revealed granular IgM in the dermo-epidermal junction. Indirect immunofluorescence was negative. Thyroid function tests showed normal thyroid stimulating hormone and free throxine4. Multinodular goiter was seen on thyroid scan and ultrasound. Thyroid fine needle aspiration was compatible with multinodular goiter (normal follicular cells, free colloid, macrophages with pigment). IV treatment with amoxicillin-clavulanic acid 1 g t.i.d. was administered for 2 weeks, with a decrease in temperature and normalization of the leukocyte level. Oral antibiotic treatment with amoxicillin-clavulanic acid was continued for 10 more days, followed by 25 days of ciprofloxacin for the osteomyelitis. Local treatment included saline soakings followed by application of Promogran (Johnson & Johnson, New Brunswick, NJ) and Kaltostat (ConvaTec Ltd., a Bristol-Myers Squibb Company, New York, NY) with slight improvement. At the same time, the patient was treated with warfarin sodium due to deep vein thrombosis under international normalized ratio 2-3. The patient was treated with IM testosterone once monthly for 1 year, which resulted in a reduction in the diameter and depth of the leg ulcers (Figure 3). Blood tests were not performed for follow-up of the immune state.
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PMID:Klinefelter's syndrome presenting with leg ulcers. 1536 65

Cervical lymphadenitis and fever are common in patients presenting to the Emergency Department (ED). Kikuchi's disease is a rare, self-limited cause of fever and cervical lymphadenitis often misdiagnosed as lymphoma or lupus and inappropriately treated, potentially causing numerous ED visits for unrelieved symptoms. The case described is that of a 29-year-old with persistent fever and cervical lymphadenitis who presented to the ED with a suspected allergic reaction to an antibiotic. The diagnosis of Kikuchi's disease was made in association with nasopharyngeal carcinoma and partial hydatidiform mole. The case highlights the clinical features, diagnosis, and treatment of Kikuchi's disease.
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PMID:Kikuchi's disease: a rare cause of cervical lymphadenitis and fever. 1602 24

Kikuchi-Fujimoto disease, also known as histiocytic necrotising lymphadenitis, is a self-limiting condition of uncertain aetiology characterised by lymphadenopathy, pyrexia, and neutropenia. Some reported cases have been associated with systemic lupus erythematosus and there have been suggestions that Kikuchi's disease could represent a mild form of lupus but without definite evidence. We describe an unusual case of histiocytic necrotising lymphadenitis in an Asian woman who had recurrent episodes for five years before a diagnosis was made.
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PMID:Recurrent Kikuchi-Fujimoto disease: case report. 1635 7

Kikuchi-Fujimoto's disease (KFD), or histiocytic necrotizing lymphadenitis, is a benign and self-limited lymphadenitis commonly found in young women. It often shares clinical features with systemic lupus erythematosus (SLE), such as arthralgias, fever and leukopenia. The etiology of KFD remains unknown and controversial. Clinical course is favorable, with spontaneous remission in less than four months in almost all cases. Herein, we present two cases. The former is a 53-year old woman presenting with cervical lymphadenopathy, arthralgia, pancytopenia and positive antinuclear antibody (ANA). Lymph node biopsy revealed histopathological features compatible with Kikuchi-Fujimoto histiocytic necrotizing lymphadenitis. The latter patient was a 20-year old woman presenting with left cervical lympadenopathy, a butterfly rash that was reminiscent of SLE, and a positive antinuclear antibody. Based upon clinical, histological and laboratory findings, the diagnosis of SLE was excluded. Careful attention should be paid to differentiating between KFD and SLE, because of their similar presentations, yet different clinical courses and therapeutic requirements.
Lupus 2006
PMID:Histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto's disease) mimicking systemic lupus erythematosus: a review of two cases. 1683 Aug 86

Kikuchi-Fujimoto's disease or histiocytic necrotizing lymphadenitis is a benign disease predominantly occurring in young women which etiology remains unknown and revealed by cervical lymphadenitis and/or prolonged fever. It has rarely been reported in children. Among the 5 cases reported, 1 child had a systemic localization. The diagnosis is based on the histological examination of a lymph node biopsy. The disease course was spontaneously favourable in 2 cases; a corticotherapy was needed in 3 children. A pathogen agent was found in 2 patients. Kikuchi's disease can reveal or evolve into autoimmune disease particularly lupus, thus a long clinical and biological follow-up is necessary.
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PMID:[Kikuchi-Fujimoto's disease: report of 5 cases and literature review]. 1793 98

Kikuchi's disease (KD) is a self-limiting histiocytic necrotizing lymphadenitis (HNL). Cutaneous manifestations are frequent and usually show histopathological findings similar to those observed in the involved lymph nodes. HNL with superposed histological features to KD has been described in patients with lupus erythematosus (LE), and a group of healthy patients previously reported as having HNL may evolve into LE after several months. Up to date, features to predict which HNL patients will have a self-limiting disease and which could develop LE have been not identified. In order to clarify the characteristics of skin lesions associated with KD, we report a case of HNL with evolution into systemic lupus erythematosus (SLE) and a review of previous reports of KD with cutaneous manifestations. A 17-year-old woman presented with a 4-month history of fever and generalised lymphadenopathy. A diagnosis of HNL was established based on a lymph node biopsy. One month later, she developed an erythematoedematous rash on her upper body, with histopathological findings of interface dermatitis. After 8 months, anti-nuclear antibodies (ANA) at titre of 1/320, anti-DNA-ds antibodies and marked decrease of complement levels were detected. During the following 2 years, she developed diagnostic criteria for SLE, with arthralgias, pleuritis, aseptic meningitis, haemolytic anaemia and lupus nephritis. To our knowledge, 27 cases of nodal and cutaneous KD have been reported, 9 of which later developed LE. In all these patients, the skin biopsy revealed interface dermatitis. Skin biopsy revealed a pattern of interface dermatitis in all reviewed KD cases, which evolved into LE. Even this histopathological finding was not previously considered significant; it might be a marker of evolution into LE.
Lupus 2008 Dec
PMID:Interface dermatitis in skin lesions of Kikuchi-Fujimoto's disease: a histopathological marker of evolution into systemic lupus erythematosus? 1902 82

Kikuchi's disease or histiocytic necrotizing lymphadenitis is a recognized cause of benign lymphadenopathy, although the current clinical literature also supports a link with systemic lupus erythematosus (SLE). Eight patients represent all the cases of Kikuchi's disease admitted to our hospital from 1990 to 1997. They were all young women of median age of 31 years (range 22-43 years). All patients showed cervical lymphadenopathy, and in two cases splenomegaly was present. In one patient, the diagnosis of lupus preceded the onset of Kikuchi's disease by 5 months. In 4 other patients, antinuclear antibodies (ANA) were positive at titers ranging between 1:80 and 1:2560 and were associated with leukopenia ranging from 1190 to 2800 white blood cells/mm3 when the diagnosis of Kikuchi's disease was made. Among these, 3 patients developed SLE after periods ranging from 6 to 25 months. The other ANA positive patient and the 2 remaining ANA negative patients did not develop any autoimmune disease after 2-A years of follow-up.The differential diagnosis between Kikuchi's disease and lupus lymphadenitis cannot always be clarified histologically. We recommend that patients with necrotizing lymphadenitis, leukopenia, and ANA be closely followed. This should ensure an early diagnosis of development of an autoimmune disorder that will require treatment, rather than always considering these patients to be affected by a benign self-limited disorder like Kikuchi's disease.
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PMID:Necrotizing Lymphadenitis in Systemic Lupus Erythematosus Kikuchi's Disease or a Kikuchi's-Like Disease? 1907 70

Histologic examination of lesions plays a key role in the diagnostics of cutaneous lupus erythematosus (LE). LE has a broad spectrum of histopathological signs, which are related to the stages of the lesions. In addition to the main subtypes of LE, we report on special manifestations like Rowell's-syndrome and Chilblain LE, and give an account of Kikuchi-Fujimoto disease (histiocytic necrotizing lymphadenitis), which may be associated with systemic LE. Furthermore the most considerable histopathologic differential diagnoses are discussed.
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PMID:Histologic features of cutaneous lupus erythematosus. 1916 43


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