Introduction:

• It is an uncommon benign disorder of lymph nodes, which usually resolves on its own in several weeks to months.
• It is also called histiocytic necrotising lymphadenitis

Epidemiology

• Common in Asian population
• Common in females
• Young adults (20-30years) are commonly affected

Etiology: Exact cause is not known

• Infections- CMV, EBV, Parvo, HHV etc
• Autoimmunity

Pathogenesis:

• Cytotoxic T lymphocytes induce apoptotic cell death and necrotising lymphadenitis

Clinical Features:

• Unilateral cervical lymphadenopathy. Other lymph nodes may be involved rarely
• Flu syndrome
  • Seen in 50% of cases
  • Fever, headache, malaise, nausea, vomiting, arthralgia, night sweats
• Maculopapular skin lesions
• Weight loss
• Rarely- Hepatosplenomegaly and aseptic meningitis

Investigations:

• Lymph node biopsy
  • Paracortical necrosis
  • Histiocytes are present along with lymphocytes
  • Karyorrhexis within macrophages which have engulfed debris
• Hemogram- Anemia, Mild granulocytopenia. Atypical lymphocytes may be seen.
• BM aspiration- Increased number of histiocytes
• ESR, CRP- May be elevated
• ANA and RA factor- Usually negative. Helps in differentiating Kikuchi disease from SLE.
• CT- To know the sites involved

Differential Diagnosis:

• SLE
• Infectious mononucleosis
• Cat scratch disease
• Kawaski disease
• Leprosy
• Sarcoidosis
• Tuberculosis
• Herpes simplex lymphadenitis
• Drug induced necrosis (Ex: phenytoin)

Pretreatment Work-up:

• History
• Examination
• Hemoglobin
• TLC, DLC
• Platelet count
• Peripheral smear
• LFT: Bili- T/D    SGPT:  SGOT:        Albumin:     Globulin:
• Creatinine
• Electrolytes: Na:        K:        Ca:          Mg:        PO4:                           
• LDH
• HIV: 
• HBsAg: 
• HCV:

Treatment: Usually only supportive treatment

• NSAIDs- To alleviate lymph node tenderness and fever
• Corticosteroids- For severe extranodal disease and for generalized marked lymphadenopathy
• Hydroxychloroquine- Useful in steroid resistant cases
• IVIg- For resistant and recurrent disease