Kimura disease is a rare chronic inflammatory disorder of unknown etiology. It usually presents as subcutaneous mass in the head and neck region and is frequently associated with regional lymphadenopathy or salivary gland involvement.It is rare in India, only 200 cases have been reported worldwide since its histopathological diagnosis.

1. Under the guidance of
Dr P.R.Modi
Dr. M.B.Mehta

2. 32 year old male patient from ahmedabad
presented with chief complaint of
swelling over left cheek since 1.5 years.
 Patient was relatively asymptomatic before 2
years when he noticed swelling over left
cheek region,
 With history of decrease and increase in the
size, with c/o ocassional itching over the
skin of that area

3.  h/o dental caries present.
 No h/o trauma
 No c/o fever, redness, pain
 No h/o any skin lesions
 No relation of size with food intake,
 No h/o any facial weakness, weight loss,
anorexia.
 No h/o chronic cough, sore throat, dry eyes,
joint pain, skin rashes.
 No h/o swelling in any other places
 No h/o allergies

4.  Past history:
No h/o DM2, Hypertension, TB, jaundice,blood
transfusion, respiratory illness
No operative history.
 Personal history:
On veg diet with normal bowel bladder
function and adequate sleep and appetite.
No addiction or drug allergies.

5. Examination
 General examination:
Pt is averagely built and nourished.
No signs of pallor, oedema, cyanosis, icterus,
lymphadenopathy

6.  Local examination:
Right side is normal.
Inspection:
A single 6*6 cm swelling is present in left
mandibular region, near the angle of
mandible, at below and front of lobule of
ear with well defined edge , and smooth
surface, with skin overlying the surface
normal.

7. Palpation:
Inspectory findings are confirmed.
A normal temperature, non tender single 6*6*3
cm, mobile, swelling of varying consistency
from soft anteriorly to firm posteriorly,
nonfluctuant, present in left mandibular
region, extending from in front of lobule of
ear and below it and below the ramus of
mandible, with well defined edge and
margin, overlying surface smooth, with
normal overlying skin normal.
sensations over the swelling normal.

8. No fixity to masseter.
On oral cavity examination:
Swelling is not bimanually palpable
No dental caries at present.
No lymph nodes palpable.
No facial weakness present.

10. So based on above history and examination
What is the diagnosis??
1. Parotitis?
2. Parotid gland swelling/tumour?
3. Submandibular Lymph node?
4. Unilateral masseter hypertrophy?

11. We went for the provisional diagnosis of
1. Parotitis
2. Parotid gland tumour.
So what are the further investigations?

12. Blood investigations:
Complete blood count:
Hb: 14.8g/dl
WBC: 18.3*10000/cumm
DC: polymorphs:60%
lymphocytes:12%
eosinophils:25%
basophils: 3%
Platelet count: 5lac/cu mm
Liver and renal function tests were normal.
Urine examination was normal.

13. USG s/o
Left parotid appears bulky and altered in echotexture
predominantly hyperechoic with internal hypoechoic,
with mildly raised internal vascularity, with intraparotid
and periparotid subcentimetric to enlarged lymph
nodes , few of them appear hypoechoic and necrotic,
findings s/o acute on chronic parotits.

14. So now what is the next line of management?
•Course of antibiotics?
•FNAC?
•CT Neck?

15. We went for FNAC
That was suggestive of eosinophils in background of
lymphocytes , with blood vessels with flat endothelial
lining, Findings p/o kimuras disease.

16. And then we went for cect neck.
s/of left parotid appears bulky, 30*30*57mm in size and
shows heterogenous enhancement with surrounding
subcentimetric lymph nodes.
Left submandibular gland appears mildly bulky as
compared to right , meauring 25*28mm.
Multiple varying size homogenously enhancing level
1a,1b,2,3 and 4 lymph nodes noted bilterally(left>right) ,
occipital lymph node on right side and level 5 on left side
with largest 16*10mm.
Subcutaneous edema and fat stranding is noted.
s/of kimuras disease..

17.  To confirm the diagnosis , we went for
Serum IgE levels , which were also
raised.(>2000)normal range is
( 0-150KIU/ml)

18. So what is the further line of management???
Conservative??
Operative??

19. Conservative management includes:
1. Observation is acceptable if the Kimura disease lesions are
neither symptomatic nor disfiguring.
2. Oral corticosteroids are commonly used; however, the disease
frequently recurs after cessation of therapy. Intralesional
corticosteroids may be effective for localized disease.
3. Cyclosporine has been reported to induce remission in patients
with Kimura disease.
4. Intravenous immunoglobulin (IVIG).
5. Oral pentoxifylline
6. All trans -retinoic acid in combination with prednisone.
7. Imatinib
8. Photodynamic therapy has been used successfully in one patient
who experienced recurrence of disease after initial surgical
management. [21]
9. Radiotherapy has occasionally been used to treat recurrent or
persistent .

20. We went for conservative management as follows:
Tablet prednesolone 40mg(1-0-0) 10 days
20mg(0-0-1)
Tablet prednesolone 40mg(1-0-0)
10mg(0-0-1) 10 days
Tablet prednesolone 40mg (1-0-0) continued for 3 months
Tablet cyclosporine 50mg(1-0-0) for 5 days
Tablet cyclosporine 50 mg (1-0-1) continued for 3 months.

21. After one month of conservative management, patient
has no complaints and size of swelling has reduced
significantly.

22. What could have been done differently?
Mass excision or superficial parotidectomy could have been
done.
However, recurrence after surgery is quite common, around 41%
Advantages:
Short duration of treatment.
Precise histopathological dignosis.

23. Differential diagnosis
1. Angiolymphoid hyperplasia with
eosinophilia,

24. Discussion
 The first known report of Kimura's disease was
from China in 1937, when Kimm and Szeto identified seven
cases of the condition.It first received its name in 1948
when Kimura and others noted a change in the surrounding
blood vessels and referred to it as "unusual granulation
combined with hyperplastic changes in lymphoid tissue.“
 It is defined as chronic eosinophillic inflammatory
condition characterised by painless, gradually growing soft
tissue mass and lymphadenopathy of the head and neck
region.
 Predominantly in asian males, age between 20 and 30 years.
 Worldwide total reported cases are 200 as per international
journal of scientific study aug 2014..vol2.


25. Other parotid swellings
 Sjogren’s syndrome
 Mikulicz disease
 Mumps parotitis
 Nonspecific parotitis

26. Conclusion
 A rare chronic inflammatory disease which
mimics neoplastic conditions. This disease
should be considered in differential
diagnosis of patients presented with head &
neck mass and lymphadenopathy and
investigated accordingly as this disease has
good prognosis
 specially in the presence of slowly
progressive swellings or any
lymphadenopathy with concomitant
eosinophilia or high-IgE levels.