Kikuchi disease
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| Micrograph of a lymph node with Kikuchi disease showing the characteristic features (abundant histiocytes, necrosis without neutrophils) on H&E stain. |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Synonyms and keywords: Histiocytic necrotizing lymphadenitis; Kikuchi-Fujimoto disease; Kikuchi necrotizing lymphadenitis; phagocytic necrotizing lymphadenitis; subacute necrotizing lymphadenitis; necrotizing lymphadenitis
Overview
Kikuchi's disease (histiocytic necrotizing lymphadenitis) is a rare, non-cancerous enlargement of the lymph nodes: the diagnosis can be confirmed by lymph node biopsy. It was originally described in Japan by Dr M. Kikuchi in 1972.[1]
Pathophysiology
Some studies have suggested a genetic predisposition to the proposed autoimmune response. Several infectious candidates have been associated with Kikuchi's disease.[2]
There are many postulates about the etiology of KFD. A microbial/viral or autoimmune cause has been suggested. Mycobacterium szulgai, Yesinia and Toxoplasma have been implicated. More recently there has been growing evidence of the role of Epstein-Barr virus, as well as other viruses (HHV6, HHV8, Parvovirus B19, HIV- and HTLV-1) in the pathogenesis of KFD. [3] But serologic tests including antibodies to a host of viruses have consistently proven noncontributory and no viral particles have been identified ultrastructurally. It is now proposed that KFD is a nonspecific hyperimmune reaction to a variety of infectious, chemical, physical and neoplastic agents. In addition to SLE, other autoimmune conditions and manifestations such as antiphospholipid syndrome, polymyositis, systemic juvenile idiopathic arthritis, bilateral uveitis, arthritis and cutaneous necrotizing vasculitis have been linked to KFD. It is possible that KFD may represent an exuberant T-cell mediated immune response in a genetically susceptible individual to a variety of non-specific stimuli. [3]
Causes
- The cause for this disease has not been established.
- Kikuchi's disease has been reported to be associated with infections such as:
Differentiating Kikuchi disease from other Diseases
Kikuchi-Fujimoto disease (KFD) is a rare, self-limiting disorder that typically affects the cervical lymph nodes. Recognition of this condition is crucial, especially because it can easily be mistaken for tuberculosis, lymphoma, or even adenocarcinoma. Awareness of this disorder will help prevent misdiagnosis and inappropriate treatment.[3]
Epidemiology and Demographics
Kikuchi's disease is a very rare disease and mainly seen in Japan. Isolated cases are reported in America, Europe and Asia. It is mainly a disease of young adults (mean age, 20–30 years), with a slight bias towards females.
Natural History
Course of the disease is generally benign and self-limiting. Lymphadenopathy most often resolves over several weeks to six months. Recurrence rate is about 3%.
Prognosis
- Kikuchi's disease is a self limiting illness.
- In most reported cases, it is common for the debilitating symptoms to last for half a year; however, the disease does return to some patients after time.
- Mortality is extremely rare and usually due to hepatic, respiratory, or cardiac failure.
Diagnosis
History
The clinical presentation is very similar symptoms to Hodgkin's lymphoma which has led to the misdiagnosis of some patients and resulting in dangerous cancer treatment to those suffering from Kikuchi's disease.
Symptoms
The symptoms of Kikuchi's are the following:
- Lymphadenopathy
- Drenching night sweats
- Fever
- Bone-joint pain
- Loss of appetite
- Unexplained weight loss
- General weakness
- Fatigue
- Mental unfocus
Laboratory Findings
It is diagnosed by lymph node excision biopsy.
Kikuchi's disease is a self limiting illness which has symptoms which may overlap with Hodgkin's lymphoma]] leading to misdiagnosis in some patients.
ANA, APLA, Anti-dsDNA, RF are usually negative, and may help in differentiating from SLE.
Treatment
- The cause for this disease has not been established, and there is also no cure.
- Kikuchi's patients are able to treat some symptoms and wait weeks to months before the symptoms of Kikuchi's has been suppressed.
Related chapters
- For other causes of lymph node enlargement, see lymphadenopathy.
References
- ↑ Kikuchi M. Lymphadenitis showing focal reticulum cell hyperplasia with nuclear debris and phagocytes. Acta Hematol Jpn 1972;35:379–80.
- ↑ Atwater AR, Longley BJ, Aughenbaugh WD (2008). "Kikuchi's disease: case report and systematic review of cutaneous and histopathologic presentations". J. Am. Acad. Dermatol. 59 (1): 130–6. doi:10.1016/j.jaad.2008.03.012. PMID 18462833. Unknown parameter
|month=ignored (help) - ↑ 3.0 3.1 3.2 Rammohan A, Cherukuri SD, Manimaran AB, Manohar RR, Naidu RM (2012). "Kikuchi-Fujimoto Disease: A Sheep in Wolf's Clothing". J Otolaryngol Head Neck Surg. 41 (3): 222–226. PMID 22762705. Unknown parameter
|month=ignored (help)
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