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 helps prevent misdiagnosis and inappropriate treatment.

Kikuchi's disease is a very rare disease mainly seen in Japan. Isolated cases are reported in North America, Europe, and Asia. It is mainly a disease of young adults (20–30 years), with a slight bias towards females. The cause of this disease is not known, although infectious and autoimmune causes have been proposed. The course of the disease is generally benign and self-limiting. Lymph node enlargmeent usually resolves over several weeks to six months. Recurrence rate is about 3%. Death from Kikuchi disease is extremely rare and usually occurs due to liver, respiratory, or heart failure.

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

Many theories exist about the cause of KFD. Microbial/viral or autoimmune causes have been suggested. "Mycobacterium szulgai" and "Yersinia" and "Toxoplasma" species have been implicated. More recently, growing evidence suggests a role for Epstein-Barr virus, as well as other viruses (HHV6, HHV8, parvovirus B19, HIV and HTLV-1) in the pathogenesis of KFD. However, many independent studies have failed to identify the presence of these infectious agents in cases of Kikuchi lymphadenopathy. In addition, serologic tests including antibodies to a host of viruses have consistently proven noncontributory and no viral particles have been identified ultrastructurally.

KFD is now proposed to be a nonspecific hyperimmune reaction to a variety of infectious, chemical, physical, and neoplastic agents. 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. KFD may represent an exuberant T-cell-mediated immune response in a genetically susceptible individual to a variety of nonspecific stimuli.

Human leukocyte antigen class II genes are more frequent in patients with Kikuchi disease, suggesting a genetic predisposition to the proposed autoimmune response.

Although it may occur in the absence of other known disease, SS is often associated with hematologic disease (including leukemia), and immunologic disease (rheumatoid arthritis, inflammatory bowel disease, Behçet's syndrome).

A genetic association has been suggested, but no specific genetic link has been identified.

SS is a reactive phenomenon and should be considered a cutaneous marker of systemic disease. Careful systemic evaluation is indicated, especially when cutaneous lesions are severe or hematologic values are abnormal. Approximately 20% of cases are associated with malignancy, predominantly hematological, especially acute myelogenous leukemia (AML). An underlying condition (streptococcal infection, inflammatory bowel disease, nonlymphocytic leukemia and other hematologic malignancies, solid tumors, pregnancy) is found in up to 50% of cases. Attacks of SS may precede the hematologic diagnosis by 3 months to 6 years, so that close evaluation of patients in the “idiopathic” group is required.

There is now good evidence that treatment with hematopoietic growth factors — including granulocyte colony-stimulating factor (G-CSF), which is used to treat AML, and granulocyte-macrophage colony-stimulating factor — can cause SS. Lesions typically occur when the patient has leukocytosis and neutrophilia but not when the patient is neutropenic. However, G-CSF may cause SS in neutropenic patients because of the induction of stem cell proliferation, the differentiation of neutrophils, and the prolongation of neutrophil survival.

Lymph node enlargement is recognized as a common sign of infectious, autoimmune, or malignant disease. Examples may include:

- Reactive: acute infection ("e.g.," bacterial, or viral), or chronic infections (tuberculous lymphadenitis, cat-scratch disease).

- The most distinctive sign of bubonic plague is extreme swelling of one or more lymph nodes that bulge out of the skin as "buboes." The buboes often become necrotic and may even rupture.

- Infectious mononucleosis is an acute viral infection caused by Epstein-Barr virus and may be characterized by a marked enlargement of the cervical lymph nodes.

- It is also a sign of cutaneous anthrax and Human African trypanosomiasis

- Toxoplasmosis, a parasitic disease, gives a generalized lymphadenopathy ("Piringer-Kuchinka lymphadenopathy").

- Plasma cell variant of Castleman's disease - associated with HHV-8 infection and HIV infection

- Mesenteric lymphadenitis after viral systemic infection (particularly in the GALT in the appendix) can commonly present like appendicitis.

Less common infectious causes of lymphadenopathy may include bacterial infections such as cat scratch disease, tularemia, brucellosis, or prevotella.

- Tumoral:

- Primary: Hodgkin lymphoma and non-Hodgkin lymphoma give lymphadenopathy in all or a few lymph nodes.

- Secondary: metastasis, Virchow's Node, neuroblastoma, and chronic lymphocytic leukemia.

- Autoimmune: systemic lupus erythematosus and rheumatoid arthritis may have a generalized lymphadenopathy.

- Immunocompromised: AIDS. Generalized lymphadenopathy is an early sign of infection with human immunodeficiency virus (HIV), the virus that causes acquired immunodeficiency syndrome (AIDS). "Lymphadenopathy syndrome" has been used to describe the first symptomatic stage of HIV progression, preceding a diagnosis of AIDS.

- Bites from certain venomous snakes such as the pit viper

- Unknown: Kikuchi disease, progressive transformation of germinal centers, sarcoidosis, hyaline-vascular variant of Castleman's disease, Rosai-Dorfman disease, Kawasaki disease, Kimura disease

"Fusobacterium necrophorum" and "Prevotella intermedia" are important bacterial pathogens in this disease process, interacting with one or more other bacterial organisms (such as "Borrelia vincentii, Porphyromonas gingivalis, Tannerella forsythia, Treponema denticola, Staphylococcus aureus", and certain species of nonhemolytic "Streptococcus").

It is often reported as a sequela to acute necrotising ulcerative gingivitis. Predisposing factors include:

- malnutrition (particularly A-and B-vitamins) or dehydration

- poor hygiene, particularly oral

- unsafe drinking water

- proximity to unkempt livestock

- recent illness

- an immunodeficiency disease, including AIDS

- measles

- smoking

It is not lethal in nature and is responsive to tetracycline or ciprofloxacin. Surgical treatment include rhinoplasty. However, if left untreated the disease can lead to sepsis, bleeding, or other chronic conditions that can be fatal.

The disease is associated with high morbidity and mortality and mainly affects children under the age of twelve in the poorest countries of Africa. Children in Asia and some countries of South America are also affected. Most children who get the disease are between the ages of two and six years old. The WHO estimates that 500,000 people are affected, and that 140,000 new cases are reported each year. The mortality rate is approximately 90 percent.

Lymphadenopathy or adenopathy is disease of the lymph nodes, in which they are abnormal in size, number, or consistency. Lymphadenopathy of an inflammatory type (the most common type) is lymphadenitis, producing swollen or enlarged lymph nodes. In clinical practice, the distinction between lymphadenopathy and lymphadenitis is rarely made and the words are usually treated as synonymous. Inflammation of the lymphatic vessels is known as lymphangitis. Infectious lymphadenitides affecting lymph nodes in the neck are often called scrofula.

The term comes from the word lymph and a combination of the Greek words , "adenas" ("gland") and , "patheia" ("act of suffering" or "disease").

Lymphadenopathy is a common and nonspecific sign. Common causes include infections (from minor ones such as the common cold to dangerous ones such as HIV/AIDS), autoimmune diseases, and cancers. Lymphadenopathy is also frequently idiopathic and self-limiting.

Kawasaki disease affects boys more than girls, with people of Asian ethnicity, particularly Japanese and Korean people, most susceptible, as well as people of Afro-Caribbean ethnicity. The disease was rare in Caucasians until the last few decades, and incidence rates fluctuate from country to country.

Currently, Kawasaki disease is the most commonly diagnosed pediatric vasculitis in the world. By far, the highest incidence of Kawasaki disease occurs in Japan, with the most recent study placing the attack rate at 218.6 per 100,000 children <5 years of age (about one in 450 children). At this present attack rate, more than one in 150 children in Japan will develop Kawasaki disease during their lifetimes.

However, its incidence in the United States is increasing. Kawasaki disease is predominantly a disease of young children, with 80% of patients younger than five years of age. About 2,000-4,000 cases are identified in the U.S. each year (9 to 19 per 100,000 children younger than 5 years of age).

In the United Kingdom, estimates of incidence rate vary because of the rarity of Kawasaki disease. However, it is believed to affect fewer than one in every 25,000 people. Incidence of the disease doubled from 1991 to 2000, however, with four cases per 100,000 children in 1991 compared with a rise of eight cases per 100,000 in 2000.

In the continental United States, Kawasaki Disease is more common during the winter and early spring, boys with the disease outnumber girls by ≈1.5–1.7:1, and 76% of affected children are <5 years of age.

More than 70% of cases are recorded in people with at least one of the following clinical situations: immunosuppression, diabetes, alcoholism/drug abuse/smoking, malignancies, and chronic systemic diseases. For reasons that are unclear, it occasionally occurs in people with an apparently normal general condition.

The infection begins locally at a site of trauma, which may be severe (such as the result of surgery), minor, or even non-apparent.

It is caused by "Klebsiella rhinoscleromatis"—subspecies of

"Klebsiella pneumoniae"— a gram-negative, encapsulated, nonmotile, rod-shaped bacillus (diplobacillus), member of the Enterobacteriaceae family. It is sometimes referred to as the "Frisch bacillus," named for Anton von Frisch who identified the organism in 1882. It is contracted directly by droplets or by contamination of material that is subsequently inhaled.

With early treatment, rapid recovery from the acute symptoms can be expected, and the risk of coronary artery aneurysms is greatly reduced. Untreated, the acute symptoms of Kawasaki disease are self-limited ("i.e." the patient will recover eventually), but the risk of coronary artery involvement is much greater. Overall, about 2% of patients die from complications of coronary vasculitis. Patients who have had Kawasaki disease should have an echocardiogram initially every few weeks, and then every one or two years to screen for progression of cardiac involvement.

Laboratory evidence of increased inflammation combined with demographic features (male sex, age less than six months or greater than eight years) and incomplete response to IVIG therapy create a profile of a high-risk patient with Kawasaki disease. The likelihood that an aneurysm will resolve appears to be determined in large measure by its initial size, in which the smaller aneurysms have a greater likelihood of regression. Other factors are positively associated with the regression of aneurysms, including being younger than a year old at the onset of Kawasaki disease, fusiform rather than saccular aneurysm morphology, and an aneurysm location in a distal coronary segment. The highest rate of progression to stenosis occurs among those who develop large aneurysms. The worst prognosis occurs in children with giant aneurysms. This severe outcome may require further treatment such as percutaneous transluminal angioplasty, coronary artery stenting, bypass grafting, and even cardiac transplantation.

A relapse of symptoms may occur soon after initial treatment with IVIG. This usually requires rehospitalization and retreatment. Treatment with IVIG can cause allergic and nonallergic acute reactions, aseptic meningitis, fluid overload and, rarely, other serious reactions. Overall, life-threatening complications resulting from therapy for Kawasaki disease are exceedingly rare, especially compared with the risk of nontreatment. Also, evidence indicates Kawasaki disease produces altered lipid metabolism that persists beyond the clinical resolution of the disease.

The cause of this disease is "Yersinia pseudotuberculosis" serotype O1. 95% are subtype O1b.

"Yersinia pseudotuberculosis" has been divided into 6 genetic groups: group 1 has only been isolated from the Far East.

Common organisms include Group A "Streptococcus" (group A strep), "Klebsiella", "Clostridium", "Escherichia coli", "Staphylococcus aureus," and "Aeromonas hydrophila", and others. Group A strep is considered the most common cause of necrotizing fasciitis.

The majority of infections are caused by organisms that normally reside on the individual's skin. These skin flora exist as commensals and infections reflect their anatomical distribution (e.g. perineal infections being caused by anaerobes).

Sources of MRSA may include working at municipal waste water treatment plants, exposure to secondary waste water spray irrigation, exposure to run off from farm fields fertilized by human sewage sludge or septage, hospital settings, or sharing/using dirty needles. The risk of infection during regional anesthesia is considered to be very low, though reported.

Vibrio vulnificus, a bacterium found in saltwater, is a rare cause.

Lymphatic disease is a class of disorders which directly affect the components of the lymphatic system.

Examples include Castleman's disease and lymphedema.

Diseases and disorder

Hodgkin's Disease/Hodgkin's Lymphoma

This is a type of cancer of the lymphatic system. It can start almost anywhere in the body. It is believed to be caused by HIV, Epstein-Barr Syndrome, age and family history. Symptoms include weight loss, fever, swollen lymph nodes, night sweats, itchy skin, fatigue, chest pain, coughing or trouble swallowing.

Non-Hodgkin's Lymphoma

Lymphoma is a usually malignant cancer. It is caused by the body producing too many abnormal white blood cells. It is not the same as Hodgkin's Disease. Symptoms usually include painless, enlarged lymph node or nodes in the neck, weakness, fever, weight loss, and anemia.

Lymphadenitis is an infection of the lymph nodes usually caused by a virus, bacteria or fungi. Symptoms include redness or swelling around the lymph node.

Lymphangitis

Lymphangitis is an inflammation of the lymph vessels. Symptoms usually include swelling, redness, warmth, pain or red streaking around the affected area.

Lymphedema

Lymphedema is the chronic pooling of lymph fluid in the tissue. It usually starts in the feet or lower legs. It's also a side-effect of some surgical procedures.

Lymphocytosis

Lymphocytosis is a high lymphocyte count. It can be caused by an infection, blood cancer, lymphoma, or autoimmune disorders that are accompanied by chronic swelling.

A similar disease is diffuse histiocytic sarcoma, a term used to designate a localized histiocytic sarcoma that has spread throughout the body.

Another disease of histiocytic origin that affects Bernese Mountain Dogs is systemic histiocytosis. This condition generally begins as lesions on the eyelids, nasal mucosa, and skin, especially the scrotum. It progresses to a more generalized disease affecting the lymph nodes, bone marrow and spleen. Other signs and symptoms include weight loss and loss of appetite. It also has a very poor prognosis.

Far East scarlet-like fever or scarlatinoid fever is an infectious disease caused by the gram negative bacillus "Yersinia pseudotuberculosis". In Japan it is called Izumi fever.

Excellent for single-focus disease. With multi-focal disease 60% have a chronic course, 30% achieve remission and mortality is up to 10%.

In pathology, dermatopathic lymphadenopathy, also dermatopathic lymphadenitis, is lymph node pathology due to skin disease.

Tuberculous lymphadenitis (or tuberculous adenitis) is a chronic specific granulomatous inflammation of the lymph node with caseation necrosis, caused by infection with "Mycobacterium tuberculosis" or "Mycobacterium bovis".

The characteristic morphological element is the tuberculous granuloma (caseating tubercule). This consists of giant multinucleated cells and (Langhans cells), surrounded by epithelioid cells aggregates, T cell lymphocytes and fibroblasts. Granulomatous tubercules eventually develop central caseous necrosis and tend to become confluent, replacing the lymphoid tissue.

Treatment with chemotherapy has been used with some success, particularly using lomustine, prednisone, doxorubicin, and cyclophosphamide. Because of the rapid progression of this aggressive disease, the prognosis is very poor.

Polyarthritis is most often caused by an auto-immune disorder such as rheumatoid arthritis, amyloidosis, psoriatic arthritis, and lupus erythematosus but can also be caused by infection with an alphavirus such as chikungunya virus and Ross River virus. This condition is termed alphavirus polyarthritis syndrome. Sindbis virus, which is endemic in Northern Europe, Africa, the Middle East, and Australia, is the most widely distributed of the alphaviruses causing polyarthritis, though infection is usually mild or asymptomatic.

Scleritis is not a common disease, although the exact prevalence and incidence are unknown. It is somewhat more common in women, and is most common in the fourth to sixth decades of life.

Incision drainage with proper evacuation of the fluid followed by anti-tubercular medication.