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Histiocytic necrotising lymphadenitis

Author: Jennifer Nguyen, Research Fellow, Victorian Melanoma Service, Alfred Hospital, VIC, Australia. DermNet NZ Editor in Chief: A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand. Copy edited by Gus Mitchell. November 2019.


What is histiocytic necrotising lymphadenitis?

Histiocytic necrotising lymphadenitis is a benign condition associated with lymphadenopathy (a disease of the lymph nodes; in this case, usually cervical), mild fever, and occasionally a rash or other systemic symptoms.

Histiocytic necrotising lymphadenitis is also known as Kikuchi disease, Kikuchi–Fujimoto disease, and histiocytic necrotising lymphadenopathy.

Who gets histiocytic necrotising lymphadenitis?

Histiocytic necrotising lymphadenitis is very rare and affects mostly Japanese and other Asians. Generally, it is more prominent in women [1–3].

What causes histiocytic necrotising lymphadenitis?

The exact cause of histiocytic necrotising lymphadenitis remains unknown. The histological and clinical presentations suggest either a viral or an autoimmune cause, which instigates a T-cell and histocyte immune response [4]. 

What are the clinical features of histiocytic necrotising lymphadenitis? 

The clinical signs of histiocytic necrotising lymphadenitis develop over two to three weeks and then subside over the next 4 months [4–6].

The hallmark of the disease is tender cervical lymphadenopathy, or rarely, lymphadenopathy affecting another region. Generalised lymphadenopathy is very rare. Mesenteric lymphadenopathy (the inflammation of the lymph nodes around the intestines) may be present.

The most common systemic signs and symptoms include:

  • Fever
  • Rash
  • Arthritis
  • Fatigue.

Other less frequent signs and symptoms are hepatosplenomegaly (swelling of the liver and spleen), weight loss, night sweats, nausea, vomiting, and diarrhoea.

What are the cutaneous manifestations of histiocytic necrotising lymphadenitis?

Transient cutaneous manifestations have been reported in up to 40% of patients with histiocytic necrotising lymphadenitis [4,5].

The cutaneous features of histiocytic necrotising lymphadenitis have included:

How is histiocytic necrotising lymphadenitis diagnosed?

Histiocytic necrotising lymphadenitis is diagnosed by lymph node biopsy. No specific blood test is available.

Elevation of erythrocyte sedimentation rate (ESR) and leukopenia have been reported [4,6].

Specific blood tests may be arranged to exclude another disease, including C3, C4, and anti-Sm to rule out SLE. 

What is the differential diagnosis for histiocytic necrotising lymphadenitis?

The differential diagnosis for histiocytic necrotising lymphadenitis includes any disorder that results in lymph node enlargement [4,6]. 

Infections should be excluded, such as:

Autoimmune disorders such as SLE need to be considered, as these may show similar clinical and histological features to histiocytic necrotising lymphadenitis. 

Lymphoma should also be considered. 

What is the treatment for histiocytic necrotising lymphadenitis?

Histiocytic necrotising lymphadenitis is self-limiting.

Symptomatic management of tender cervical lymphadenopathy may include paracetamol and non-steroidal anti-inflammatory drugs. Patients with a severe clinical course or relapsing disease may be treated with oral corticosteroids [4,6].

What is the outcome for histiocytic necrotising lymphadenitis?

Histiocytic necrotising lymphadenitis lasts for one to four months. The recurrence rate is 3–4% [4,6].

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Related information

 

References

  1. Bosch X, Guilabert A, Miquel R, Campo E. Enigmatic Kikuchi-Fujimoto disease: a comprehensive review. Am J Clin Pathol 2004; 122: 141–52. PubMed
  2. Dorfman RF, Berry GJ. Kikuchi's histiocytic necrotizing lymphadenitis: an analysis of 108 cases with emphasis on differential diagnosis. Semin Diagn Pathol 1988; 5: 329–45. PubMed
  3. Lin HC, Su CY, Huang CC, Hwang CF, Chien CY. Kikuchi's disease: a review and analysis of 61 cases. Otolaryngol Head Neck Surg 2003; 128: 650-3. PubMed
  4. Perry AM, Choi SM. Kikuchi-Fujimoto Disease: A Review. Arch Pathol Lab Med 2018; 142: 1341–6. PubMed
  5. Kucukardali Y, Solmazgul E, Kunter E, Oncul O, Yildirim S, Kaplan M. Kikuchi–Fujimoto disease: analysis of 244 cases. Clin Rheumatol 2007; 26: 50–4. PubMed
  6. Bosch X, Guilabert A. Kikuchi-Fujimoto disease. Orphanet J Rare Dis 2006; 1: 18. doi:10.1186/1750-1172-1-18. PubMed Central

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