myself dr.khan , MD PATHOLOGY , GOLD MEDALIST , BLOGGER , YOU TUBER, with the aim of creating precise notes for all exam going students of pathology (MBBS/BDS/MD RESIDENTS/DNB RESIDENTS/FELLOWS/ NEET ASPIRANTS) and also for senior consultants who struggle every day to learn something new to update themselves with recent advances in the subject

2. KIKUCHI DISEASE

3. DEFINITION
• Benign disease characterized by lymphadenopathy with distinctive
histologic features.

4. SYNONYMS
KIKUCHI
FUJIMOTO
DISEASE
KIKUCHI
LYMPHADENITIS
HISTIOCYTIC
NECROTIZING
LYMPHADENITIS
PHAGOCYTIC
NECROTIZING
LYMPHADENITIS
SUBACUTE
NECROTIZING
LYMPHADENITIS.

5. EPIDEMIOLOGY
Generally affects young Asian female with average age at presentation
27years.

6. ETIOLOGY
• unknown

7. CLINICAL FEATURES

8. Unilateral tender cervical lymphadenopathy
Fever
rash
Aneamia
neutropenia
rarely patients may present with atypical lymphocytosis.

9. DIAGNOSIS
• Lymph node biopsy

10. PATHOGENESIS
• NOT clear.
• Recent evidences show that extensive karyorrhectic debri is due to
early apoptosis of CD 8 T lymphocytes.

11. LIGHT MICROSCOPY
1. Patchy Lymph node involvement , usually centered in paracortical
areas.
2. Atleast occasional crescentic histiocytes .
3. Fibrinoid necrosis surrounded by immunoblasts , histiocytes and
plasmacytoid dendritic cells.
4. Extracellular apoptotic/karyorrhectic debri.
5. Absence of eosinophils,polymorphonuclear cells and plasma cells.

12. MORPHOLOGICAL TYPES OF KIKUCHI

13. • Predominance of foamy histiocytes , and
necrosis can be present or absent.
XANTHOMATOUS TYPE
• Predominantly phagocytic histiocytes with
single cell necrosis
PHAGOCYTIC TYPE
• Crescentic histiocytes, immunoblasts ,
plasmacytoid dendritic cells with
apoptosis but without coagulative
necrosis.
PROLIFERATIVE TYPE
• Similar to PROLIFERATIVE type with
additional large areas of necrosis
NECROTIZING TYPE

14. RESIDUAL FOLLICLE

15. EXTENSIVE NECROSIS

17. IMMUNOHISTOCHEMISTRY

18. Lymphoid cells are
T cells, often with
numerous cytotoxic
(CD8+) cells
Histiocytes express
myeloperoxidase,
lysozyme , CD68.
B cells are rare to
absent in and
around the necrotic
foci.

19. DIFFERENTIAL DIAGNOSIS

21. ELECTRON MICROSCOPY

22. PROGNOSIS AND MANAGEMENT
PROGNOSIS
• Few patients develop recurrent lymphadenopathy
• Rare patients, usually with an underlying immunodeficiency , have a
severe illness with a poor outcome.
THERAPY- nearly all patients have a self limited illness requiring no
specific therapy.

23. CONCLUSION
• Benign disease with spontaneous remission common in young
women involving unilateral post cervical LN with preserved lymph
node architecture , necrosis surrounded by histiocytes and shows
abundant karryorhectic debri , absence of neutrophils is a must to
diagnose this entity.

24. QUICK RECAP
• DEFINITION
• SYNONYMS
• EPIDEMIOLOGY
• CLINICAL FEATURES
• PATHOGENESIS
• HISTOLOGICAL TYPES
• DIAGNOSIS
• IMMUNOHISTOCHEMISTRY
• DIFFERENTIAL DIAGNOSIS
• ELECTRON MICROSCOPY
• PROGNOSIS
• MANAGEMENT