Kimura disease, also known historically as eosinophilic hyperplastic lymphogranuloma, is a rare benign inflammatory disease that characteristically manifests as enlargement of cervical lymph nodes and salivary glands.
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Epidemiology
Kimura disease typically affects males (80%) between 20-40 years of age (80% of cases) 1,2, and is most frequently seen in Asia. Sporadic cases are seen in other geographic regions, however these are uncommon. An infectious agent is presumed to be the cause of an immunological response, however no specific pathogen has as yet been identified 4,5.
Clinical presentation
Presentation is with subcutaneous masses and enlargement of the lymph nodes of the head and neck, particularly near the angle of the mandible and postauricular groups. Salivary glands (particularly the parotid and submandibular glands) and lymph nodes of the axilla, groin, popliteal fossa, medial epicondyle 6 and elsewhere may also be involved 1,4,5. They are usually not painful. Involvement of adjacent soft tissues is uncommon, although direct extension into the pinna of the ear has been described.
These changes are usually associated with eosinophilia in the peripheral blood and in tissues, and marked increase in serum levels of immunoglobulin E (IgE) 4,5.
There have been case reports of eosinophilic panniculitis 7 in patients with Kimura disease and some authors have reported presentations with nephrotic syndrome 8.
Pathology
Microscopic appearance
Histologically Kimura disease is characterized by:
proliferation of lymphoid follicles
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cellular infiltrates
eosinophils (majority), sometimes progressing to eosinophilic abscesses
also plasma cells lymphocytes, and mast cells
vascular proliferation of post-capillary venules
fibrosis
Peripheral eosinophilia is common.
Radiographic features
Ultrasound
Ultrasound is a useful modality for assessment of the neck and to aid in biopsy. Sonographic features include 2:
solid, enlarged nodes sometimes maintaining hilar architecture
hypoechoic, usually homogeneous: ~90%
foci of necrosis uncommon: ~15%
increased vascularity usually in a hilar distribution: ~90%
salivary glands also hypoechoic, but usually more heterogeneous
CT
CT demonstrates non-specific appearances of:
marked enlargement of cervical nodes +/- parotid and submandibular glands
intense enhancement of nodes
heterogeneous enhancement of major salivary glands
MRI
MRI signal intensity and enhancement varies depending on the amount of fibrosis and vascular proliferation present 1.
T1: hypointense or isointense compared with salivary tissue
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T2
typically hyperintense compared to salivary gland tissue
variable according to degree of fibrosis
T1 C+ (Gd): usually homogeneous enhancement
Treatment and prognosis
Kimura disease is benign, and no firmly established treatment protocols have been described. Options include 4,5:
conservative management
radiotherapy
steroid and oxyphenbutazone (with only transient improvement)1
resection
cyclosporine
Recurrence rate is high for steroids and surgery, and as such radiotherapy is favored by some authors 4, although as the condition is benign and patients are young, not all agree 1. There were seven patients reportedly cured with cyclosporine 8.
History and etymology
It was first described in China by Kim and Szeto in 1937 as eosinophilic hyperplastic lymphogranuloma 9. It was then described in 1948 by T Kimura et al. in Japan 5,10, after whom the disease is named, an illustration of Stigler's law.
Differential diagnosis
Differential diagnosis on imaging is that of lymph node enlargement, and includes:
benign reactive nodes / infectious mononucleosis / drug reactions
nodal metastases
parasitic infections
Histologically Kimura disease was initially thought to be related to angiolymphoid hyperplasia with eosinophilia (ALHE), however clinically and radiologically these two entities are clearly different:
ALHE affects primarily White females
ALHE involves dermis without lymph node enlargement
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