Kikuchi's disease, also known as histiocytic

Histiocyte

A histiocyte is a cell that is part of the mononuclear phagocyte system . The mononuclear phagocytic system is part of the organism's immune system...

 necrotizing

Necrosis

Necrosis is the premature death of cells in living tissue. Necrosis is caused by factors external to the cell or tissue, such as infection, toxins, or trauma. This is in contrast to apoptosis, which is a naturally occurring cause of cellular death...

 lymphadenitis and Kikuchi-Fujimoto disease, is a rare, non-cancerous enlargement of the lymph node

Lymph node

A lymph node is a small ball or an oval-shaped organ of the immune system, distributed widely throughout the body including the armpit and stomach/gut and linked by lymphatic vessels. Lymph nodes are garrisons of B, T, and other immune cells. Lymph nodes are found all through the body, and act as...

s.

It was first described in Japan by Dr Masahiro Kikuchi in 1972 and independently by Y. Fujimoto.

Epidemiology

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 preponderance towards females. The cause of this disease is not known although infectious and autoimmune

Autoimmune disease

Autoimmune diseases arise from an overactive immune response of the body against substances and tissues normally present in the body. In other words, the body actually attacks its own cells. The immune system mistakes some part of the body as a pathogen and attacks it. This may be restricted to...

 etiologies have been proposed. 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%. Mortality is extremely rare and usually due to hepatic, respiratory, or cardiac failure.

Pathophysiology

Some studies have suggested a genetic predisposition to the proposed autoimmune response. Several infectious candidates have been associated with Kikuchi's disease, including cytomegalovirus

Cytomegalovirus

Cytomegalovirus is a viral genus of the viral group known as Herpesviridae or herpesviruses. It is typically abbreviated as CMV: The species that infects humans is commonly known as human CMV or human herpesvirus-5 , and is the most studied of all cytomegaloviruses...

, Epstein Barr virus, Herpes simplex virus

Herpes simplex virus

Herpes simplex virus 1 and 2 , also known as Human herpes virus 1 and 2 , are two members of the herpes virus family, Herpesviridae, that infect humans. Both HSV-1 and HSV-2 are ubiquitous and contagious...

, Varicella zoster virus

Varicella zoster virus

Varicella zoster virus is one of eight herpes viruses known to infect humans . It commonly causes chicken-pox in children and Herpes zoster in adults and rarely in children.-Nomenclature:...

, parainfluenza virus, parvovirus B19

Parvovirus B19

The B19 virus, generally referred to as parvovirus B19 or sometimes erythrovirus B19, was the first known human virus in the family of parvoviruses, genus erythrovirus...

 and paramyxovirus

Paramyxovirus

Paramyxoviruses are viruses of the Paramyxoviridae family of the Mononegavirales order; they are negative-sense single-stranded RNA viruses responsible for a number of human and animal diseases.-Genera:*Subfamily Paramyxovirinae**Genus Avulavirus Paramyxoviruses (from Greek para-, beyond, -myxo-,...

. Association between systemic lupus erythematosus

Systemic lupus erythematosus

Systemic lupus erythematosus , often abbreviated to SLE or lupus, is a systemic autoimmune disease that can affect any part of the body. As occurs in other autoimmune diseases, the immune system attacks the body's cells and tissue, resulting in inflammation and tissue damage...

 (SLE) and Kikuchi's disease is not proved.

Clinical Features

The signs and symptoms of Kikuchi's disease are fever

Fever

Fever is a common medical sign characterized by an elevation of temperature above the normal range of due to an increase in the body temperature regulatory set-point. This increase in set-point triggers increased muscle tone and shivering.As a person's temperature increases, there is, in...

, lymphadenopathy

Lymphadenopathy

Lymphadenopathy is a term meaning "disease of the lymph nodes." It is, however, almost synonymously used with "swollen/enlarged lymph nodes". It could be due to infection, auto-immune disease, or malignancy....

, skin rashes and headache

Headache

A headache or cephalalgia is pain anywhere in the region of the head or neck. It can be a symptom of a number of different conditions of the head and neck. The brain tissue itself is not sensitive to pain because it lacks pain receptors. Rather, the pain is caused by disturbance of the...

. Rarely hepatosplenomegaly and nervous system involvement resembling meningitis

Meningitis

Meningitis is inflammation of the protective membranes covering the brain and spinal cord, known collectively as the meninges. The inflammation may be caused by infection with viruses, bacteria, or other microorganisms, and less commonly by certain drugs...

 is seen.
Differential diagnosis includes SLE, disseminated tuberculosis

Tuberculosis

Tuberculosis, MTB, or TB is a common, and in many cases lethal, infectious disease caused by various strains of mycobacteria, usually Mycobacterium tuberculosis. Tuberculosis usually attacks the lungs but can also affect other parts of the body...

, lymphoma

Lymphoma

Lymphoma is a cancer in the lymphatic cells of the immune system. Typically, lymphomas present as a solid tumor of lymphoid cells. Treatment might involve chemotherapy and in some cases radiotherapy and/or bone marrow transplantation, and can be curable depending on the histology, type, and stage...

, Sarcoidosis

Sarcoidosis

Sarcoidosis , also called sarcoid, Besnier-Boeck disease or Besnier-Boeck-Schaumann disease, is a disease in which abnormal collections of chronic inflammatory cells form as nodules in multiple organs. The cause of sarcoidosis is unknown...

, and viral lymphadenitis.Clinical findings sometimes may include positive results for IgM/IgG/IgA Antibodies.

For other causes of lymph node enlargement, see lymphadenopathy

Lymphadenopathy

Lymphadenopathy is a term meaning "disease of the lymph nodes." It is, however, almost synonymously used with "swollen/enlarged lymph nodes". It could be due to infection, auto-immune disease, or malignancy....

.

Diagnosis

It is diagnosed by lymph node excision biopsy

Biopsy

A biopsy is a medical test involving sampling of cells or tissues for examination. It is the medical removal of tissue from a living subject to determine the presence or extent of a disease. The tissue is generally examined under a microscope by a pathologist, and can also be analyzed chemically...

.

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

Rheumatoid factor

Rheumatoid factor is an autoantibody most relevant in rheumatoid arthritis. It is defined as an antibody against the Fc portion of IgG. RF and IgG join to form immune complexes that contribute to the disease process...

are usually negative, and may help in differentiating from SLE.

Management

No specific cure.
Treatment largely supportive. NSAIDs for tender lymphnodes and fever, corticosteroids are useful in severe extranodal or generalized disease.