This book describes Kikuchi Disease, Diagnosis and Treatment and Related Diseases

Kikuchi-Fujimoto disease, also known as histiocytic necrotizing lymphadenitis, is a very rare benign (non-cancerous) disorder of the lymph nodes.

Kikuchi-Fujimoto disease was first described in Japan in 1972, as a benign and self-limiting disease that mainly involves young women of Asian origin.

Most patients are younger than 30 years of age, but this disorder has been reported in patients varying in age from six to 80 years, most of whom were well previously.

The mean age at manifestation in a USA series was 30 years.

Kikuchi disease mostly involves young adults (younger than 30 years) and is most often found in East Asian and Japanese populations but rare in the United States and Europe.

This geographic link can be related to certain HLA alleles such as HLA class II alleles, HLA-DPA1, and HLA-DPB1, frequent in Asian Kikuchi disease patients and rare in Caucasians.

Kikuchi disease can involve both males and females.

The ratio of involved males to females in 3 series was 1:4, 1:1.6, and 1:1.26, respectively.

Kikuchi disease has since been found in all racial ethnic groups and many countries e.g., the USA.

In a study of patients with Kikuchi disease in the USA, 75 % were Caucasian.

The incidence of this disorder differs widely in different groups, but it has been most often reported from Asia.

While the precise cause of Kikuchi-Fujimoto disease is not known, infectious and autoimmune causes have been indicated.

Several infectious micro-organisms have been indicated as possible causative agents, herpes simplex virus type 1 and 2, varicella-zoster virus, cytomegalovirus, Epstein-Barr virus (EBV), parvovirus B19, human papillomavirus, human herpesvirus (HHV 6, 7, 8)hepatitis B virus, human T-lymphotropic virus 1, Brucella, toxoplasma, and Yersinia.

Kikuchi-Fujimoto disease has an autoimmune cause induced by virus-infected transformed lymphocytes and is also linked with systemic lupus erythematosus.

This is because histopathological findings in the lymphocytes and the endothelial cells in patients with systemic lupus erythematosus are similar to those found in patients with Kikuchi disease

Kikuchi disease is now suggested to be a nonspecific hyper-immune reaction to a range of infectious, chemical, physical, and neoplastic agents.

Other autoimmune disorders and presentations such as antiphospholipid syndrome, polymyositis, systemic juvenile idiopathic arthritis, bilateral uveitis, arthritis and cutaneous necrotizing vasculitis have been linked to Kikuchi disease.

The main symptoms of Kikuchi disease are:
1. Enlargement of the lymph nodes (lymphadenopathy),
2. Skin rashes,
3. Mild fever,
4. Headache and
5. Night sweats.

Less frequent symptoms are:
1. Weight loss,
2. Nausea,
3. Vomiting, and
4. Sore throat.

Lymphadenopathy features are:
1. Cervical nodes are involved in about 80% of cases
2. Posterior cervical nodes are often involved (70% of cases)
3. Lymphadenopathy is isolated to a single location in 83% of cases, but multiple chains may be involved
4. Less often affected nodes are in axillary, mediastinal, celiac, abdominal, and inguinal sites

Medically, Kikuchi disease appears like a lot of other diseases, and the differential is broad such as reactive lymphadenitis, viral infections, tuberculosis, systemic lupus erythematosus, lymphoma, and metastasis.

The gold standard diagnostic test for Kikuchi disease is the histopathological analysis of excised lymph node biopsy specimen.

Kikuchi disease is...