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Kimura, morbusL98.9
Synonym(s)
Kimura syndrome; Kimura Syndrome
HistoryThis section has been translated automatically.
Kimura, 1948
DefinitionThis section has been translated automatically.
Multicentre, benign lymphoproliferative hyperplasia, characterised by subcutaneous nodules with oedema, lymphocytic and eosinophilic infiltrates, formation of germinal centres, fibrosis and hyperplasia of blood vessels. Apparently, there are differences between Kimura disease and angiolymphoid hyperplasia with eosinophilia due to clinical and histological criteria, so that the two clinical pictures can be managed as separate entities. See below Angiolymphoid hyperplasia with eosinophilia.
Occurrence/EpidemiologyThis section has been translated automatically.
Mainly in the Asian population, less frequently among Caucasians and Africans.
EtiopathogenesisThis section has been translated automatically.
Unknown.
ManifestationThis section has been translated automatically.
Men are affected significantly more often than women; the peak age is between 20 and 30 years. Occasionally, cases of the disease have also been described in children and adolescents, mostly in connection with nephrotic syndrome or dialysis.
LocalizationThis section has been translated automatically.
Head and neck region.
Clinical featuresThis section has been translated automatically.
Single or multiple, 2-40 mm in size, hemispherical, red to reddish-brownish, more rarely purple or ulcerated cutaneous or subcutaneous nodules and nodes. Rarely disseminated occurrence or lymphadenopathy. No itching.
LaboratoryThis section has been translated automatically.
Significant blood eosinophilia (> 10%); increased IgE.
HistologyThis section has been translated automatically.
Subcutaneously localized, diffuse infiltrate of T- and B-lymphocytes and numerous eosinophils, isolated eosinophilic microabscesses, moderate fibrosis, slight edema, formation of numerous germinal centres, increased occurrence of blood vessels with epithelioid endothelials, numerous, partly solid vascular sprouts from poorly differentiated cell elements, single mast cells.
DiagnosisThis section has been translated automatically.
Due to the uncharacteristic clinic, the diagnosis is made exclusively via biopsy.
Differential diagnosisThis section has been translated automatically.
TherapyThis section has been translated automatically.
Excision for longer periods of storage. Danger of bleeding! Recurrence treatment (but also applied as primary therapy) with fractionated x-ray radiation (GD: 20-40 Gy). Also combination of intralesional glucocorticoid application like triamcinolone acetonide crystal suspension (e.g. Volon A) diluted 1:3 with a local anaesthetic (e.g. scandicain) are described as successful.
Internal therapyThis section has been translated automatically.
Test with Ciclosporin A over a period of 1/2 year.
LiteratureThis section has been translated automatically.
- Hareyama M et al (1998) Radiotherapy for Kimura's disease: the optimum dosage. Int J Radiat Oncol Biol Phys 40: 647-651
- Helander SD et al (1995) Kimura`s disease and angiolymphoid hyperplasia: New observations from immunohistochemical studies of lymphocyte markers, endothelial antigens, and granulocyte proteins. J Cutan catheter 22: 319-326
- Hung CC et al (2000) Steroid-sensitive anemia in a boy on dialysis - an association with Kimura disease. Pediatric Nephrol 15: 183-185
- Kaneko K et al (1999) Successful tratment of Kimura's disease with cyclosporine. J Am Acad Dermatol 41: 893-894
- Khoo BP, Chan R (2002) Kimura disease: 2 case reports and a literature review. Cutis 70: 57-61
- Kimura T et al (1948) Unusual granulation combined with hyperplastic changes of lymphoid tissue. Trans Soc Pathol Jpn 37: 179
- Krapohl BD et al (2003) A rare vasoproliferative lesion: angiolymphoid hyperplasia with eosinophilia of the hand. Br J Plast Surgery 56: 168-170
- Lu HJ (2003) Kimura disease in a patient with renal allograft failure secondary to chronic rejection. Pediatric nephrol 18: 1069-1072
- Metze D et al (1991) Angiolymphoid hyperplasia with eosinophilia (AHE). Dermatologist 42: 101-106