Kikuchi Disease Clinical Presentation

Updated: Sep 25, 2018
  • Author: John Boone, MD; Chief Editor: Koyamangalath Krishnan, MD, FRCP, FACP  more...
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Presentation

History

Kikuchi disease most frequently manifests as a relatively acute onset of cervical adenopathy associated with fever and a flulike prodrome.

Lymphadenopathy features are as follows:

  • Cervical nodes are affected in about 80% of cases
  • Posterior cervical nodes are frequently involved (65-70% of cases)
  • Lymphadenopathy is isolated to a single location in 83% of cases, but multiple chains may be involved
  • Cases of generalized adenopathy involving axillary, inguinal, and mesenteric nodes are unusual

A flulike prodrome with fever is present in 50% of cases. The following are less common signs and symptoms:

  • Headache
  • Nausea, vomiting
  • Malaise, fatigue
  • Weight loss
  • Arthralgias, myalgias
  • Night sweats
  • Rash (up to 30%)
  • Thoracic/abdominal pain
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Physical

Characteristics of lymphadenopathy are as follows:

  • Lymphadenopathy is isolated to a single location in 83% of patients, although multiple nodal chains may be involved

  • Cervical nodes are affected in 80% of patients; of these, 65-70% involve posterior triangle cervical nodes

  • Less commonly affected nodes include those in axillary, mediastinal, celiac, abdominal, and inguinal locations

  • The nodes are usually described as painless or mildly tender

  • The nodes tend to be 2-3 cm in diameter, although masses of multiple nodes may reach 6 cm

  • The nodes are usually firm and mobile, but they are not fluctuant or draining

Extranodal findings are as follows:

  • Skin [26, 27] : The incidence of skin involvement varies from 5-30%; findings are varied and nonspecific and include maculopapular lesions, morbilliform rash, nodules, urticaria, and malar rash, which may resemble that of systemic lupus erythematosus (SLE); skin lesions resolve in a few weeks to months

  • Hepatosplenomegaly: This finding is not uncommon; monitor lactate dehydrogenase (LDH) levels

  • Neurologic involvement: Neurologic involvement is rare but has included conditions such as aseptic meningitis, acute cerebellar ataxia, and encephalitis [28] ; patients with aseptic meningitis may report headache, but they do not exhibit nuchal rigidity or positive Kernig or Brudzinski signs; cerebrospinal fluid (CSF) findings are similar to those noted in patients with aseptic meningitis of viral etiology

  • Rarely involved extranodal sites include the bone marrow, myocardium, uvea, and thyroid and parotid glands

  • Arthritic involvement: Asymmetric polyarthritis, enthesitis, and dactylitis of the toes was reported in the case of a 14-year-old boy [29]

  • Widespread involvement of multiple organ systems in Kikuchi disease has been described in solid-organ transplant patients

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