IgD Immune Complex Vasculitis in a Patient With Hyperimmunoglobulinemia D and Periodic Fever

Boom, Bart W.

doi:10.1001/archderm.1990.01670360085015

Cited by 41 publications

(11 citation statements)

References 14 publications

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“…Reasoning from the clinical phenotype, we chose to investigate apoptosis of lymphocytes in HIDS. Lymphadenopathy, lymphocytic infiltrates in skin biopsies, and many B lymphocytes containing IgD in the cytoplasm in bone marrow can be observed in HIDS, 11,12 and this may be related to the lymphocyte apoptosis defect. In the other periodic fever syndromes, different cell types are more prominent and point to cell-specific defects.…”

Section: Resultsmentioning

confidence: 99%

Defective apoptosis of peripheral-blood lymphocytes in hyper-IgD and periodic fever syndrome

Bodar

Hilst

Heerde³

et al. 2006

Blood

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Hereditary periodic fever syndromes are characterized by incapacitating attacks of fever and generalized inflammation. While the mutated genes for the major syndromes in this group are known, the pathogenesis remains unclear. The aim of this study was to investigate apoptosis in patients with periodic fever as a possible pathogenic factor. We measured anisomycin-induced apoptosis with annexin-V flow cytometry and caspase-3/7 activity in peripheral-blood lymphocytes from symptom-free patients with hyper-IgD and periodic fever syndrome (HIDS; n ‫؍‬ 10), TNF-receptor-associated periodic syndrome (TRAPS; n ‫؍‬ 7), and familial Mediterranean fever (FMF; n ‫؍‬ 2). HIDS lymphocytes showed a decreased percentage of apoptosis during remission by both methods compared with controls (17.8% vs 55.4%), whereas no difference was observed in TRAPS or FMF lymphocytes. This defective apoptosis of lymphocytes may be a central pathogenic mechanism in HIDS, since dysfunction of one of the inhibitory mechanisms to curtail the immunologic response could cause an unbridled generalized inflammation after a trivial stimulus.

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Section: Resultsmentioning

confidence: 99%

Defective apoptosis of peripheral-blood lymphocytes in hyper-IgD and periodic fever syndrome

Bodar

Hilst

Heerde³

et al. 2006

Blood

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“…Very recently, mutations in the mevalonate kinase gene were shown to cause HIDS (41,64). It has been proposed that patients exhibit an uncontrolled type III hypersensitivity reaction, possibly with involvement of IgD-containing complexes (16). Complement deficiencies or decreases in serum C3 and C4 were not found, albeit circulating immune complexes were detected in 20% of patients during or between attacks (36).…”

Section: Immunodeficienciesmentioning

confidence: 99%

Immunoglobulin D: Properties, Measurement, and Clinical Relevance

Vladutiu

2000

Clin Diagn Lab Immunol

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“…In addition, a number of other conditions have been associated with elevated serum IgD levels, including IgD multiple myeloma, Hodgkin disease, cigarette smoking, pregnancy, immunodeficiency syndromes, and recurrent infections. 9,47,48 Furthermore, some individuals with typical findings of HIDS and mutations in MVK do not have elevated serum IgD levels. 34 New laboratory methods, such as genetic testing for MVK or biochemical analysis of urine for elevated mevalonic acid, may be better to establish or confirm the diagnosis of HIDS.…”

Section: Commentmentioning

confidence: 99%

“…Similarly, cutaneous lesions are common in HIDS. [47][48][49][50] In a study, 47 79% of patients with HIDS had skin lesions dur-…”

Section: Commentmentioning

confidence: 99%

Tumor Necrosis Factor Receptor–Associated Periodic Syndrome

Toro

Aksentijevich²,

Hull³

et al. 2000

Arch Dermatol

131

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Background: Tumor necrosis factor receptorassociated periodic syndrome (TRAPS) is an inflammatory disorder characterized by prolonged episodes of periodic fever and localized inflammation and dominantly inherited mutations in TNFRSF1A, the gene encoding the 55-kDa tumor necrosis factor receptor. To our knowledge, the cutaneous pathologic characteristics of TRAPS have not been described previously.Objectives: To characterize the dermatologic manifestations of TRAPS by clinical, microscopic, and molecular methods, and to investigate its immunophenotype.Design, Setting, and Patients: At the National Institutes of Health Clinical Center, Bethesda, Md, a tertiary care referral center, 25 patients with a clinical and molecular diagnosis of TRAPS were evaluated clinically and 10 biopsy specimens of lesional skin were examined by light microscopy and immunohistochemistry. Patients were screened for mutations in TNFRSF1A, the gene coding for the p55 tumor necrosis factor receptor.Main Outcome Measures: Clinical, light microscopic, and immunohistochemical features. Results:The skin eruption usually lasted 4 to 21 days (mean, 13 days). Of 25 patients, 21 (84%) presented with migratory erythematous macules and patches and 10

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IgD Immune Complex Vasculitis in a Patient With Hyperimmunoglobulinemia D and Periodic Fever

Cited by 41 publications

References 14 publications

Defective apoptosis of peripheral-blood lymphocytes in hyper-IgD and periodic fever syndrome

Defective apoptosis of peripheral-blood lymphocytes in hyper-IgD and periodic fever syndrome

Immunoglobulin D: Properties, Measurement, and Clinical Relevance

Tumor Necrosis Factor Receptor–Associated Periodic Syndrome

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