Fever and Lymphadenopathy

Posted by Sara Fazio • December 13th, 2013

In the latest Case Record of the Massachusetts General Hospital, a 30-year-old man was admitted to the hospital because of fever, chills, and lymphadenopathy that developed 6 months after he returned from a trip to India. Imaging revealed diffuse lymphadenopathy in the neck and chest and splenomegaly. Diagnostic tests were performed.

Localized lymphadenopathy is common, and 55% of all cases involve lymph nodes in the head and neck, including the cervical region. In contrast, 1% of all cases of lymphadenopathy are attributed to supraclavicular lymph nodes, 5% to axillary nodes, and 14% to inguinal nodes.

Clinical Pearls

• What is Kimura’s disease?

Kimura’s disease, which is often confused with angiolymphoid hyperplasia with eosinophilia, is a rare, benign, chronic inflammatory disorder of unknown cause that mainly affects young Asian men. Patients with Kimura’s disease have an indolent presentation, with painless lymphadenopathy in the head and neck. It is common to see eosinophilic infiltrates in the biopsy specimens.

• What is Kikuchi-Fujimoto disease?

Kikuchi-Fujimoto disease, which was first described in 1972, is seen in young Asian persons. The clinical features include symptom onset over a period of 2 to 4 weeks, fevers, lymphadenopathy (usually cervical), leukopenia (in less than or equal to 58% of cases, according to the original description of the disease by Kikuchi), respiratory symptoms, arthralgias and myalgias, and skin manifestations (e.g., ulcers, erythematous plaques, and papules). Fine-needle aspiration biopsy of a lymph node is not a reliable diagnostic test for Kikuchi-Fujimoto disease; the aspirate usually shows crescentic histiocytes, karyorrhectic and granular debris, and plasmacytoid monocytes.

Morning Report Questions

Q: What is the prognosis of Kikuchi-Fujimoto disease?

A: Generally, Kikuchi-Fujimoto disease resolves without treatment in less than 6 months, although the use of glucocorticoids and such antibiotics as ciprofloxacin and minocycline may result in improvement. Recurrences have been reported but are rare, regardless of treatment modality; death due to the disease is also rare, occurring in approximately 2% of patients.

Q: What condition is histologically closest to Kikuchi-Fujimoto disease, leading to possible misdiagnosis?

A: SLE [systemic lupus erythematosus]-related lymphadenitis is the condition histologically closest to Kikuchi-Fujimoto disease. Hematoxylin bodies (basophilic condensations of nuclear and cytoplasmic debris) and the Azzopardi effect (which occurs when nuclear debris adheres to the surface of blood vessels) are findings specific to SLE-related lymphadenitis, but they are uncommon. In patients with SLE-related lymphadenitis, the lymph nodes tend to contain more neutrophils and plasma cells than do those in patients with Kikuchi-Fujimoto disease; the presence of vasculitis outside necrotic areas is also more common in patients with SLE-related lymphadenitis. SLE may occasionally develop in patients months or years after the findings on a lymph-node biopsy suggested Kikuchi-Fujimoto disease.

6 Responses to “Fever and Lymphadenopathy”

  1. Maria Virginia Bosco says:

    very good case!

  2. Prof. Dr. Gerd-Michael Lackmann, MD says:

    In my opinion, both rare diseases under discussion, Kimura’s disease and Kikuchi-Fujimoto disease, are not well diagnosed. Although lymph node biopsy may be the only hard diagnostic criterium for differential diagnosis, clinical aspects should also be considered. So, the patient under discussion present neither respiratory symptoms nor arthralgias/myalgias nor skin manifestations, all characteristic symptoms of Kikuchi-Fujimoto disease. Furthermore, it is stated in the discussion of this case itself, that fine – needle lymph node aspiration biopsy is not a reliable diagnostic test for this rare disorder.
    Therefore, I suggest that other diseases must be considered in differential diagnosis: SLE, as mentioned above; any kind of autoimmune vasculitis with con committed lymphadenopathy (vascular biopsy and histological as well as immunohistochemical investigations), Hodgkin- and Non – Hodgkin lymphomas (lymph node biopsy), CML or CLL (bone marrow aspiration) and infectious agents (multiple serological testings, including parasites and fungi).

  3. dagoberto rodriguez martinez says:

    very very good for the clinical excercise i remember my school practices at speciality clinical patology.tanks for the information

  4. Indran Muthiah says:

    Can malignancies,especially Lung,present this way.?

  5. Dr R K Shenoy says:

    Head and Neck is the commonest site for isolated adenopathy by the sheer presence of maximum no of nodes. Three hundred nodes are present in the neck out of a 800 in the whole body.

  6. Jason says:

    I have Kimura’s disease, this guy almost certainly does not :). To diagnose Kimura’s disease you mostly only need too look at the tumour with an ultrasound and do a white cell count and IgE levels test. That is all. If you want to be 100% sure you haven’t missed something you can do a biopsy of the tumour, CT, MRI, bone marrow biopsies. But in my honest opinion, you don’t need to do any of that once you have seen one case and know how it looks and mostly how reactive it is. None of the other conditions have tumours that can grow 5 times larger overnight and then go back to their “small size”. On the ultrasound you should see one vascular tumour and one enlarged normal node under it. Then a string of borderline large nodes. There is no fever or chills even with 40% eosinophilia, only a persistent itching skin