Kikuchi-Fujimoto disease with multiple extra-nodal features - A clinical mimic

Area of Science:

• Rheumatology
• Pathology
• Internal Medicine

Background:

• Kikuchi's disease (KD) is a rare, benign lymphohistiocytic disorder.
• Extranodal involvement in KD is exceptionally uncommon.

Observation:

• A previously healthy 31-year-old Indian woman presented with high-grade fever, arthralgia, and rash.
• Clinical presentation included erythematous maculopapular rash, symmetric polyarthritis, and hepatosplenomegaly.
• Diagnostic workup revealed negative anti-nuclear antibodies and characteristic features of KD on lymph node biopsy.

Findings:

• The patient exhibited multiple extranodal manifestations of Kikuchi's disease.
• Lymph node biopsy was crucial for diagnosing KD and excluding other conditions.
• The case underscores the diagnostic challenges posed by extranodal KD.

Implications:

• Extranodal KD can mimic hematological malignancies, connective tissue diseases, and infections.
• Accurate differentiation from systemic lupus erythematosus (SLE) is critical.
• Lymph node biopsy remains the gold standard for definitive KD diagnosis.