KIMURA DISEASE

NTRODUCTION

A chronic inflammatory disorder  of uncertain etiology with distinctive histologic features mainly affecting the head and neck that appears to represent an aberrant immunologic reaction characterized by eosinophilia , increased serum IgE and angiolymphoid proliferation.

SYNONYMS

Kimura lymphadenopathy , Eosinophilic lymphogranuloma

INCIDENCE uncommon

GENDER,RACE, AND AGE DISTRIBUTION

Most patients are young adult males, Asians are more commonly affected than whites or blacks.

ETIOLOGY –unknown.

 

CLINICAL FEATURES

Patients typically have  painless unilateral or bilateral cervical lymphadenopathy.

One or more  slowly growing , large , painless lesions in subcutaneous and deep soft tissue which may persist or recur over a period of months or years.

Some patients may have proteinuria , nephrotic syndrome or asthma.

MORPHOLOGY

Lesions generally poorly demarcated.

Lymph nodes and other involved sites show an infiltrate of lymphocytes, eosinophils, plasma cells, mast cells, reactive follicles, and fibrosis.

Eosinophilic abscesses and polykaryocytes, especially in follicles.

Collagen deposition may be seen , on long standing  diffuse hyalinization changes may be seen.

EXTENSIVE SUBCUTANEOUS INVOLVEMENT BY KIMURA DISEASE IN A YOUNG MALE - LOW POWER VIEW

SCANNER VIEW SHOWING REACTIVE FOLLICLES WITH EOSINOPHILS WITHIN THEM.

LOW POWER VIEW SHOWING HYPERPLASTIC FOLLICLE WITH NUMEROUS EOSINOPHILS WITH IN AND AROUND THE FOLLICLE

HIGH POWER VIEW SHOWING PREDOMINANCE OF EOSINOPHILS IN KIMURA LYMPHADENOPATHY

IMMUNOPHENOTYPE

Ig E positive follicular dendritic networks in follicles.

Vascular endothelial cells – factor VIII , Ulex europaeus agglutinin (UEA-1).

DIAGNOSIS

Biopsy of involoved tissue

DIFFERNETIAL DIAGNOSIS

 

PROGNOSIS AND THERAPY

The lymphadenopathy and other mass lesions do not require specific therapy , although large and persistent can be excised.

 

CONCLUSION

Chronic inflammatory disease of uncertain etiology , commonly seen in young Asian males with involvement of lymphnodes , salivary glands and kidneys, hyperplastic germinal centers with abundant eosinopilic infiltration  involving germinal centers and microabscess ,peripheral blood eosinophilia and increased IgE levels.

EXTRA EDGE

https://youtu.be/mhsNWo6Lwm8I