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1/94. Kikuchi-Fujimoto's syndrome masquerading as tuberculosis.

    We report a case of a 27-year-old Asian man presenting with the typical features of tuberculous cervical lymphadenitis who was commenced on anti-tuberculous therapy on the strength of the clinical presentation. Histological examination of an excised cervical lymph node however, revealed the diagnosis of Kikuchi's syndrome; a histiocytic necrotizing lymphadenitis which is usually self-limiting. ( info)

2/94. histiocytic necrotizing lymphadenitis (Kikuchi's disease) with aseptic meningitis.

    histiocytic necrotizing lymphadenitis, or Kikuchi's disease (KD), is a self-limited clinicopathologic entity recognized increasingly worldwide. A 27-year-old man with cervical lymphadenopathy and fever who was diagnosed with KD developed mild headache with no nuchal rigidity. The cerebrospinal fluid (CSF) was sterile and contained 78 white blood cells/mm3 with lymphocytes predominating, accompanied by smaller numbers of monocytes and granulocytes. This abnormality normalized spontaneously over 5 weeks. Eleven similar cases have been reported, all but one from japan. The development of meningitis in KD was observed in four (9.8%) of 41 KD patients we have treated, suggesting that the meningitis was related to KD and not merely coincidental. ( info)

3/94. histiocytic necrotizing lymphadenitis (Kikuchi's disease): the necrotic appearance of the lymph node cells is caused by apoptosis.

    We report a case of histiocytic necrotizing lymphadenitis in a 28-year-old woman. The biopsy specimen of the enlarged lymph node showed lymphocytes, histiocytes, and a large amount of nuclear debris as well as marked eosinophilic deposits. We found dna fragments by means of the modified TUNEL method, especially in the transitional area between intact cells and the foci of eosinophilic deposits and the cells positive for anti-Fas antibody in the biopsied lymph node. Therefore, the necrotic appearance of the lymph node was thought to be caused by apoptosis induced by the Fas-Fas ligand system. We hypothesize that the apoptosis was strongly related to the pathogenesis of this disease. ( info)

4/94. Kikuchi's disease with multisystemic involvement and adverse reaction to drugs.

    Kikuchi's disease (KD), or histiocytic necrotizing lymphadenitis, was initially described in japan in 1972. In the following years, several series of cases involving patients of different ages, races, and geographic origins were reported, but pediatric reports have been rare. The etiology of KD is unknown, although a viral or autoimmune hypothesis has been suggested. The most frequent clinical manifestation consists of local or generalized adenopathy, although in some cases, it is associated with more general symptoms, multiorganic involvement, and diverse analytic changes (leukopenia, elevated erythrocyte sedimentation rate, and c-reactive protein, as well as an increase of transaminases and serum lactic dehydrogenase). diagnosis is based on characteristic pathologic findings that permit differentiation of this disease from lymphoma, systemic lupus erythematosus, and infectious lymphadenopathies. We present here the case of a 14-year-old boy who presented with severe systemic manifestations and transient fulminant hepatic failure in response to treatment with antituberculosis drugs. Kikuchi's disease, lymphadenitis, liver failure, antituberculosis drugs. ( info)

5/94. The histopathology of cutaneous lesions of Kikuchi's disease (necrotizing lymphadenitis): a report of five cases.

    Kikuchi's disease (KD) is an idiopathic, self-limited necrotizing lymphadenitis that can clinically and histologically mimic high-grade lymphoma, including Hodgkin's disease, or can be mistaken for the lymphadenitis of systemic lupus erythematosus (SLE). Involvement of extranodal sites is unusual but well documented, especially in asia, where KD is more common than in north america or europe. The successful distinction of KD from malignant lymphoma and SLE is imperative for the appropriate treatment of affected patients. We describe five patients with cutaneous involvement by KD, all of whom presented with fever, lymphadenopathy, and an eruption on the skin of the upper body, which in one case was clinically suspected to be due to SLE and in another, polymorphous light eruption. The patients ranged in age from 10 months to 42 years (median, 33 years) and included three females and two males. All five patients had negative serologic studies for collagen vascular disease. Each patient had a lymph node biopsy showing the typical necrotizing lymphadenitis of KD. skin biopsies from all five patients shared a specific constellation of histologic features: vacuolar interface change with necrotic keratinocytes, a dense lymphohistiocytic superficial and deep perivascular and interstitial infiltrate, varying amounts of papillary dermal edema, and abundant karyorrhectic debris with a conspicuous absence of neutrophils and a paucity of plasma cells, paralleling the nodal histology in KD. CD68 immunohistochemistry on paraffin-embedded sections showed many histiocytes and plasmacytoid monocytes in all cases, whereas CD3, CD4, and CD8 showed highly variable staining among the cases. There was only rare staining with TIA-1 and CD30. We believe that the papular eruption of KD has recognizable histopathologic features and that a CD68 stain that marks many cells that initially seem to be lymphocytes can be performed to confirm the diagnosis. ( info)

6/94. Kikuchi disease in systemic lupus erythematosus: clinical features and literature review.

    Kikuchi disease (histiocytic necrotizing lymphadenitis) is a rare, benign entity which predominantly affects young women. The disease usually presents with painful or painless enlarged cervical lymph nodes accompanied with fever. The cause of the disease is uncertain and only 17 cases in the literature have been reported in the past to be associated with systemic lupus erythematosus (SLE). We report four cases of SLE with Kikuchi disease in taiwan. This is the first report regarding Kikuchi disease and SLE in taiwan. All patients are female. The mean age is 30 (ranging from 21 to 35 years). The mean history of SLE is 4 years (ranging from 10 days to 8 years). Three of our patients (75%) developed Kikuchi disease accompanied with flare-up of lupus activity, and the other one had Kikuchi disease simultaneously with the onset of SLE. One patient had ribosomal-P antibodies, one had ribonuclear protein (RNP) antibodies and none had antibodies to Ro(SSA) or La (SSB). The cause of association of Kikuchi disease with SLE is still unknown. From our cases, the high frequency of flare-up of lupus disease activity accompanied with the onset of Kikuchi disease and the simultaneous occurrence of these two diseases indicate that they are not independent events. We speculate that Kikuchi disease may be one of the manifestation of SLE. ( info)

7/94. histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto disease) diagnosed by fine needle aspiration biopsy.

    A case of histiocytic necrotizing lymphadenitis involving a right submandibular lymph node is described in a 14-year-old Caucasian boy. The diagnosis was established on initial assessment by fine needle aspiration biopsy. Excisional biopsy, performed because the lymph node enlargement failed to regress, confirmed the cytological findings. ( info)

8/94. Kikuchi's disease: an uncommon cause of fever of unknown origin.

    Kikuchi's disease or histiocytic necrotizing lymphadenitis is an uncommon clinical condition that causes diagnostic difficulties because of its lack of specific signs, symptoms and serological markers. diagnosis is based on histopathological findings, but overlapping of the histological features requires a differential diagnosis among histiocytic necrotizing lymphadenitis and a number of infectious, autoimmune and lymphoproliferative lymphadenopathies. We report a case of Kikuchi's disease in a 19-year-old woman presenting with generalized lymphadenopathy and persistent fever. ( info)

9/94. Kikuchi-Fujimoto lymphadenitis with cutaneous involvement.

    We report a case of Kikuchi-Fujimoto disease with cutaneous involvement in a European man. In contrast, the disease is most prevalent in women of Asian descent. The condition is probably under-recognized when histology is not used to identify lymphadenitis of protracted course. skin lesions may mimic clinically other unrelated disorders including lymphomas and immune or infectious dermatoses. histology of skin lesions may bring a clue to the diagnosis when apoptotic plasmacytoid monocytes are recognized. The relationship between Kikuchi-Fujimoto disease and lupus erythematous remains a matter of debate. ( info)

10/94. Fine needle aspiration cytology in systemic lupus erythematosus lymphadenopathy. A case report.

    BACKGROUND: Systemic lupus erythematosus (SLE) is an autoimmune disease of unknown etiology characterized by inflammation in various organ systems, including lymph nodes, due to the production of antinuclear antibodies. The onset of disease is between ages 13 and 40 years, with a female preponderance. CASE: A 30-year-old female presented with right cervical lymphadenopathy and gave a history of intermittent fever and swollen joints of 2.5 years' duration. The patient was on intermittent corticosteroids. With a suggestion of tuberculous lymphadenitis, the patient underwent fine needle aspiration (FNA). The diagnosis of lupus adenopathy was established by FNA of enlarged right cervical lymph nodes. Smears showed predominantly typical and atypical immunoblasts, plasma cells, occasional Reed-Sternberg-like cells and dispersed hematoxylin bodies. Smears were negative for acid-fast bacilli. CONCLUSION: When SLE patients develop lymphadenopathy, FNA cytology helps differentiate lupus adenopathy from infectious conditions, such as tuberculous adenitis, and from Kikuchi's lymphadenitis. ( info)
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