Kikuchi-Fujimoto Disease Masquerading as Acute Appendicitis

Abstract

Kikuchi-Fujimoto Disease (KFD) is a self-limiting necrotizing lymphadenitis that usually presents with fever and cervical lymphadenopathy. Recognition of this condition is crucial, because it can be mistaken for tuberculosis, lymphoma and connective tissue disorders. When present at an unusual location the diagnosis can be challenging. We present an unusual case of Kikuchi-Fujimoto disease involving mesenteric lymph node masquerading as acute appendicitis along with its differential diagnosis. A 30-year-old female presented with complaints of acute abdominal pain, vomiting and fever. Physical examination revealed rebound tenderness in the right iliac fossa. The abdominal sonography was suspicious of acute appendicitis. The patient underwent appendectomy with excision of an enlarged mesenteric lymph node. On histopathology mesenteric node showed features of KFD which was confirmed on immunohistochemistry. Appendix was unremarkable. Although rare KFD should be added to the differential diagnosis of acute appendicitis in patients with enlarged mesenteric lymph nodes, Awareness of this disorder helps to prevent misdiagnosis and inappropriate treatment.

Acute fulminant necrotizing mesenteric lymphadenitis causing bowel ischemia

“…Two were diagnosed during surgery for presumed appendicitis, and one by biopsy. KFD has a classic histiocytic necrotizing inflammatory process within the lymph nodes, which can be confirmed by immunohistochemistry [8]. But none of those patients had disease so extensive as to cause mesenteric ischemia, and the patient in this report did not have immunohistochemistry consistent with KFD.…”

Bilateral preretinal hemorrhage associated with Kikuchi-Fujimoto disease

“… 13 , 14 The classical presentation of KFD includes fever and enlargement of cervical lymph nodes, though other lymph nodes may also be involved. Other manifestations include gastrointestinal pain (varies from simple epigastric pain to appendicitis-like pain), 15 , 16 , 17 maculopapular rash, night sweats, anemia, and leukopenia, as well as relative lymphocytosis with atypical lymphocytes. 13 , 14 , 18 , 19 …”

Kikuchi-Fujimoto Syndrome: A Rare Entity to Consider

“…KFD can be even more challenging to diagnose when there is ganglion involvement not localized to the cervical region. Cases of acute abdomen resulting from this pathology have been described, and its presentation as pyelonephritis, as described above, is unprecedented [ 1 ] .…”

“…Kikuchi-Fujimoto disease (KFD)is a rare, benign, necrotizing lymphadenitis of unknown aetiology, with fever and preferential involvement of the cervical region [ 1 ] . This self-limiting condition may, due to its similarity, be confused with tuberculosis, systemic lupus erythematosus and haematological malignancies [ 2 , 3 ] .…”

“…No aetiological factors have been identified, but it is thought that this syndrome results from an immune response by CD8+ T-lymphocytes and histiocytes to an infectious insult [ 1 – 3 ] . The main aetiological cause is assumed to be viral [ 3 ] .…”