Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We have encountered 2 patients with mixed connective tissue disease (MCTD) associated with lymphadenopathy at the outset. Biopsy specimens of enlarged lymph nodes showed massive necrotizing lesions with follicular hyperplasia, which are occasionally observed in the involved lymph nodes of patients with systemic lupus erythematosus (SLE) and called lupus lymphadenitis. Usually, the only histologic finding of an involved lymph node in MCTD is lymphoid hyperplasia. These are the 2nd and 3rd cases of MCTD associated with lupus lymphadenitis. Our cases suggest that histologic studies of the swollen lymph nodes are not helpful in distinguishing MCTD from SLE.
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PMID:Mixed connective tissue disease associated with lupus lymphadenitis. 844 Nov 49

A 9-year-old Chinese girl with systemic lupus erythematosus presented initially with persistent cervical lymphadenopathy without any constitutional symptoms. The histology of the involved lymph node showed histiocytic necrotizing lymphadenitis and the initial clinical diagnosis of Kikuchi-Fujimoto disease was made. The patient subsequently developed fever, oral ulcerations, epistaxis, generalised myalgia and erythema multiforme-like skin lesions over the face and upper arms. Investigations showed anaemia, leucopenia, hypocomplementemia, a positive antinuclear antibody and a positive anti-double-stranded DNA antibody test. The clinical diagnosis was revised to systemic lupus erythematosus. The histological picture of histiocytic necrotizing lymphadenitis can occur in systemic lupus erythematosus and may be indistinguishable from that seen in Kikuchi-Fujimoto disease. The diagnosis of Kikuchi-Fujimoto disease should be confined to a benign clinical subset of histiocytic necrotizing lymphadenitis which resolves spontaneously without treatment. The occurrence of erythema multiforme-like lesions in lupus erythematosus is unusual and together with the characteristic serologic abnormalities, fits Rowell's syndrome.
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PMID:Systemic lupus erythematosus with erythema multiforme-like lesions and histiocytic necrotizing lymphadenitis--a case report. 889 39

Histiocytic necrotizing lymphadenitis (Kikuchi's disease) is an uncommon disease of the cervical lymph nodes occurring in young women, commonly associated with various auto-immune or infectious diseases. We describe the case of a 17 year-old girl who presented a Kikuchi's lymphadenitis occurring concomitantly with Hashimoto's thyroiditis and lupus serology as anti-nuclear, anti-DNA, anticardiolipid antibodies and hypocomplementemia. The patient was treated with prednisone and hydroxychloroquine. Thirty months after, she is doing well and hydroxychloroquine is continued.
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PMID:[Kikuchi syndrome, Hashimoto thyroiditis and lupus serology. Apropos of a case]. 897 77

The Kikuchi-Fujimoto's syndrome is an histiocytic necrotizing lymphadenitis which is observed at any ages but preferentially among young adults. The sex ratio is about 4:1 in favour of women. It is clinically characterized by cervical lymph nodes in a context of fever and asthenia. Sometimes, there is transitory leucopenia and an increase of the erythrocyte sedimentation rate. The course of the disease is spontaneously favourable in 1 or 3 months but recurrence is possible. The histology of the lymph node could mimic a malignant lymphoma and the immunohistochemical findings are of a great importance (Ki-M1P or KP1 antibody). The etiology remains unknown but some infectious diseases have been suspected (toxoplamosis, Epstein-Barr virus). Its association with a systemic lupus erythematous had been described and this set the problem of its physiopathology. We report two new cases of Kikuchi-Fujimoto's syndrome which one was attributed to Epstein-Barr virus primo-infection and the other associated with a systemic lupus erythematous.
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PMID:[Kikuchi-Fujimoto necrotizing histiocytic lymphadenitis: apropos of 2 cases and review of the literature]. 897 79

We report one case of a cervical pseudo-tumoral form of histiocytic necrotizing lymphadenitis (Kikuchi Fugimoto disease) occurring in a 13-year-old child. Diagnosis was made only by histology examination of an excision biopsy from one cervical lymph node. Spontaneous complete resolution occurred within 2 months. A review of the clinical and histological features in adults and children is presented. No difference was found between the two populations regarding these features. Differential diagnoses are malignant lymphoma, systematic lupus erythematosus and, in particular, Still's disease in children. The etiology of the disease is also discussed.
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PMID:A pseudo-tumoral form of Kikuchi's disease in children: a case report and review of the literature. 980 13

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.
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PMID:Kikuchi disease in systemic lupus erythematosus: clinical features and literature review. 1049 56

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.
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PMID:Kikuchi-Fujimoto lymphadenitis with cutaneous involvement. 1056 91

A registry of United States residents with chronic granulomatous disease (CGD) was established in 1993 in order to estimate the minimum incidence of this uncommon primary immunodeficiency disease and characterize its epidemiologic and clinical features. To date, 368 patients have been registered; 259 have the X-linked recessive form of CGD, 81 have 1 of the autosomal recessive forms, and in 28 the mode of inheritance is unknown. The minimum estimate of birth rate is between 1/200,000 and 1/250,000 live births for the period 1980-1989. Pneumonia was the most prevalent infection (79% of patients; Aspergillus most prevalent cause), followed by suppurative adenitis (53% of patients; Staphylococcus most prevalent cause), subcutaneous abscess (42% of patients; Staphylococcus most prevalent cause), liver abscess (27% of patients; Staphylococcus most prevalent cause), osteomyelitis (25% of patients; Serratia most prevalent cause), and sepsis (18% of patients; Salmonella most prevalent cause). Fifteen percent of patients had gastric outlet obstruction, 10% urinary tract obstruction, and 17% colitis/enteritis. Ten percent of X-linked recessive kindreds and 3% of autosomal recessive kindreds had family members with lupus. Eighteen percent of patients either were deceased when registered or died after being registered. The most common causes of death were pneumonia and/or sepsis due to Aspergillus (23 patients) or Burkholderia cepacia (12 patients). Patients with the X-linked recessive form of the disease appear to have a more serious clinical phenotype than patients with the autosomal recessive forms of the disease, based on the fact that they are diagnosed significantly earlier (mean, 3.01 years of age versus 7.81 years of age, respectively), have a significantly higher prevalence of perirectal abscess (17% versus 7%), suppurative adenitis (59% versus 32%), bacteremia/fungemia (21% versus 10%), gastric obstruction (19% versus 5%), and urinary tract obstruction (11% versus 3%), and a higher mortality (21.2% versus 8.6%).
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PMID:Chronic granulomatous disease. Report on a national registry of 368 patients. 1084 35

Histiocytic necrotizing lymphadenitis or Kikuchi-Fujimoto disease is a rare entity, and even more rarely, it is associated with other diseases. In a few cases, the condition is associated with cutaneous lupus erythematosus (CLE). Histiocytic necrotizing lymphadenitis predominantly affects young women, who present with solitary or multiple cervical lymphadenopathy accompanied by symptoms such as fever, weight loss, sweating, or, in exceptional cases, hepatosplenomegaly. Laboratory examinations show normal or nonspecific results. The disease is of unknown origin, although a viral origin has been suggested, with the suspected agents including Epstein-Barr virus, herpesvirus type 6, and cytomegalovirus (CMV). Although the first and most of the more recent cases have been reported in Oriental patients, the disease has a wide geographic distribution. The clinical evolution is favorable, with spontaneous remission in less than 4 months in almost all cases. We present a case of a 37-year-old woman from Peru who presented with cervical adenopathies on two occasions. Biopsy of a lymph node revealed a histopathologic picture compatible with Kikuchi-Fujimoto histiocytic necrotizing lymphadenitis. The adenopathy disappeared in a few months. A year later, she presented with a maculopapular rash in the nasal and malar regions. The results of the skin biopsy and immunofluorescence examination were compatible with chronic CLE. The results of the serology testing for CMV were positive. Treatment with chloroquine was initiated, with almost complete recovery by 5 months. No manifestations of systemic lupus erythematosus have occurred since. The epidemiologic, clinical, and anatomopathologic aspects as well as the differential diagnosis of this entity are reviewed.
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PMID:Kikuchi-Fujimoto necrotizing lymphadenitis associated with cutaneous lupus erythematosus: a case report. 1094 58

Kikuchi's disease is a histologically alarming self-limiting condition typically affecting the lymph nodes of young females. A 13-year-old girl was presented with fever, skin rash and cervical lymphadenopathy. On examination she was found febrile, mild pallor was present and she had lymphadenopathy. Liver was palpable. Cervical lymph node biopsy showed histiocytic necrotising lymphadenitis (Kikuchi's disease). Dengue virus serology for IgG blot showed evidence of seroconversion in serial samples. She was treated with antibiotics and fluconazole and cyclosporin A. During hospitalisation she developed retinal vasculitis. She was reviewed after one month and showed rashes of subacute cutaneous lupus erythematosus. This case can be described to be a triggering event by dengue viral infection causing abnormal immune response leading initially to Kikuchi's disease and later on to systemic lupus erythematosus.
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PMID:Unusual association of Kikuchi's disease and dengue virus infection evolving into systemic lupus erythematosus. 1563 44


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