Clinical Immunology and Immunopathology
Brief CommunicationKimura's Disease with High Serum Levels of Eosinophil Cationic Protein and Major Basic Protein
Abstract
This study reports a case of Kimura's disease. The patient was a 58-year-old man and satisfied the clinico-pathological findings of Kimura's disease. Serum eosinophil cationic protein (ECP) and major basic protein (MBP) levels in this patient were also measured. In the active stage of the disease, the patient's serum ECP and MBP levels were higher than those of healthy controls and declined in the remission stage. This finding suggests that ECP and MBP may be important agents in the development of Kimura's disease.
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Serum concentrations of eosinophil cationic protein and eosinophils of patients with Kimura's disease
2007, Allergology InternationalBackground: To clarify the role of eosinophils in the pathogenesis of Kimura's disease and the values of measuring serum levels of eosinophil cationic protein (ECP) for monitoring disease activity might be very important, but there are few reports about this matter.
Methods: A total 14 serum and 7 tissue samples from patients with Kimura's disease were studied. The concentrations of ECP and cytokines (interleukin-4 (IL-4), granulocyte-macrophage colony-stimulating factor (GM-CSF), and interleukin 5 (IL-5)) in sera from patients with Kimura's disease were measured by enzyme-linked immunosorbent assay (ELISA). The density of eosinophils and the degree of activation of eosinophils in the tissue were also studied immunohistochemically.
Results: The concentration of ECP in sera from patients with Kimura's disease was significantly higher than that in the control group (p < 0.05). At the time of the remission, a significant decrease of ECP was observed. In interfollicular areas, most infiltrated eosinophils were positive for EG2 antibody (64.0-94.0%) and the mean percentage of EG2-positive eosinophils was 75.7%. The concentrations of IL-4, gM-CSF, and IL-5 in sera from patients with Kimura's disease were within normal ranges or below the detectable level in all sera examined.
Conclusions: Our findings suggest that eosinophils play an important role in the pathogenesis of Kimura's disease and ECP may be used as an additional parameter of disease activity.
Kimura's disease of the parotid glands
2004, Asian Journal of Oral and Maxillofacial SurgeryKimura's disease is an idiopathic, chronic inflammatory disorder predominantly affecting the subcutaneous tissues of the head and neck. Most patients have involvement of the major salivary glands and regional lymph nodes with associated eosinophilia and an elevated immunoglobulin E concentration. This report presents a patient with Kimura's disease involving the parotid glands, and includes a review of the literature.
Treatment of Kimura's disease by interferon and corticosteroid
1999, Revue de Medecine InterneNous rapportons un cas de maladie de Kimura survenue chez un enfant caucasien âgé de 5 ans après une morsure de tique dans la région cervicale. Le diagnostic a été posé chez l'enfant alors âgé de 16 ans devant un tableau associant de volumineuses adénopathies cervicales et occipitales à une hyperéosinophilie (1 640/mm3) et un taux d'IgE élevé (18 866 KU/L). Nous présentons l'effet clinique etimmunologique d'un traitement associant interféron-α et corticoïdes généraux et discutons son intérêt.
L'examen anatomopathologique d'une adénopathie a montré une hyperplasie lymphoïde folliculaire et vasculaire avec présence de nombreux polynucléaires éosinophiles et maslocytes. Il a été noté la présence d'un fort contingent de cellules CD4+, CD27−, CD7∈n faveur d'un profil TH2. La production d'IL-5 était augmentée et inhibée in vitro par les interférons α et β. Le bilan étiologique est resté négatif. La responsabilité d'antigènes provenant de Ixodes ricinus dans l'initiation de l'affection n'a pas été démontrée. Compte tenu du caractère évolutif des adénopathies et de l'échec de trois cures chirurgicales, un traitement par interféron-α (Introna®, Schering-Plough, 5.106 U/semaine) et bolus d'hemisuccinate de méthylprédnisolone (1 g/mois) a été instauré. Il a entraîné la régression des adénopathies, sans modification de l'éosinophilie, ni du taux d'eosinophil cationic protein (ECP) avec un taux d'IgE restant élevé. La diminution de la corticothérapie a entraîné ne nouvelle augmentation du volume des adénopathies avec élévation du taux d'ECP.
Il s'agit du premier cas de maladie de Kimura traité par interféron-α. L'évolution n'indique pas que l'efficacité de l'interferon-α soit prédominante.
A case of Kimura's disease that occurred in a 5-year-old Caucasian boy after a tick bite is reported. When the child was 16 vears old, symptoms developed. They included voluminous bilateral neck and head lymph nodes associated with hypereosinophilia (1,640/mm3), and increased IgE levels (18,866 KU/L). Clinical and immunological effects of treatment by interferon-α and steroids are presented.
Pathological and histological examination showed typical features of dense lymphoid cell infiltrates containing many eosinophils, cells, and vascular hyperplasia. Percentages of CD4+, CD27−. CD7lls were increased In the blood and lymph nodes, showing a profile typical of TH2. IL-5 production by these cells was markedly increased and was inhibited by IFN-α and IFN-β in vitro. No etiology was found. The role of antigens of Ixodes ricinus is discussed. Three surgical excisions of adenopathies were not successful. Treatment by IFN-α.(Introna®, Schering-Plough, 5.106 U/week) and a bolus of inethylprednisolone hemisuccinate (1 g/month) was started. Eight months later, the size of the lymph nodes had decreased; however, eosinophil counts and ECP and IgE levels were still high. The decrease in corticosteroid induced a subsequent, slight increase in the size of the lymph nodes and a marked increase in ECP.
This is the first description of treatment of Kimura's disease by interferon-α and steroids. The disease outcome does not suggest that inlerferon-α may predominate.
Rare hyper-immunoglobulin E syndromes
1998, Revue de Medecine InterneLe dosage des immunoglobulines E totales est fréquemment demandé, en particulier par les allergologues. Leur augmentation est classiquement retrouvée au cours de l'atopie et des parasitoses. Le but de cet article est d'énumérer de rares pathologies où les immunoglobulines E totales se trouvent augmentées en dehors des contextes classiques.
Ces rares hyperimmunoglobulinémies E sont pour la majorité liées à un déficit immunitaire plus ou moins sévère, responsable d'infections virales, fungiques et bactériennes.
Le syndrome de Buckley: infections profondes à staphylocoques dorés, dermatose chronique. La maladie de Netherton: atteinte cutanéophanérienne, manifestations allergiques. Le syndrome de Wiskott-Aldrich: thrombopénie et thrombopathie, dermatose. La maladie de Di-George: hypoparathyroiïdie, infections. Le déficit sélectif en immunoglobuline A: infections récidivantes. Le syndrome de Buckley et la maladie de Netherton qui associent des manifestations cutanées eczématiformes et une augmentation des immunoglobulines E totales sont parfois difficiles à différencier de la dermatite atopique.
Total immunoglobulin E levels are very frequently measured, particularly for allergy. Their high levels are ordinarily found in atopy and parasitosis. This article subject is to determine rare diseases where high levels of total immunoglobuline E are found except in classic disease.
These rare hyper-immunoglobulin E syndromes are more often associated with immunologic deficiency more or less intense that lead to viral, fungal and bacterial infections.
Buckley syndrome: deep infections due to Staphylococcus aureus, chronic dermatitis. Netherton disease: cutaneous and hair disease, allergic manifestations. Wiskott-Aldrich syndrome: thrombocytopeny and -pathy, dermatitis. Di-George disease: hypoparathyroidism, infections. Selective deficit of immunoglobulin A: recurrent infections.
It is difficult to differentiate between atopic dermatitis and Buckley syndrome and Netherton disease because of eczematiform chronic dermatitis and high-levels of total immunoglobulin E found in all these diseases.
Diagnostic challenges and updated therapeutic strategies of Kimura's disease: A case report successfully treated by dupilumab and review
2023, Medicine (United States)Kimura's disease: Effects of age on clinical presentation
2020, QJM: An International Journal of Medicine