Kimura’s Disease: Symptoms and Treatment

Introduction:

Kimura's disease was previously recognized as "eosinophilic hyperplastic lymphogranuloma" when it was first described in 1937 in Chinese literature by HT Kimm and C Szeto. The disease bears the author's name because Kimura et al. presented the authoritative histological description of the condition in 1948. The frequency of reports of the sickness has gradually increased since that point.

What Is Kimuras Disease?

Kimura's illness is an uncommon condition that often manifests as non-painful subcutaneous swellings in the head and neck, typically in the pre-auricular (in front of the auricle of the ear) and submandibular regions (area below the lower jaw). It is accompanied by

• Severe peripheral eosinophilia (an increase in eosinophils, a type of white blood cells that support the immune system by fighting against infection).

• Increased IgE levels (a protein the immune system produces in response to a potential invasion).

• Local and distant lymphadenopathy (swelling of the lymph nodes).

Who Gets the Disease?

• Most instances of Kimura disease (80 % of cases) involve males between 20 and 40 years.

• It is most common in East and Southeast Asia, with a limited number of cases reported in Europe and the Middle East.

• Kimura disease can strike children, but it is relatively uncommon, and cases have been documented in infants as young as 15 months.

• Middle-aged people have been diagnosed with Kimura disease most frequently in the Far East, including China and Japan. Although sporadic occurrences occasionally occur in other parts of the world, they are rare.

What Causes the Disease?

It needs to be clarified how the condition develops and what causes it.

• The prevailing opinion is that Kimura disease develops due to a delayed hypersensitivity reaction. The lymphocytes' unique cell membrane features serve as antibodies to bind foreign antigens. The contact between lymphocytes and antigens, such as viruses or tissues from their dermis, holds this together.

• Sensitized lymphocytes alter morphologically and biochemically during the antigen-antibody response. Through target cells, which in this instance are skin cells, they get the capacity to enter the region where the immunocomplex reaction occurs. Inflammation that is both productive and proliferative occurs along with this.

• As the inflammation worsens, it impairs vascular permeability, tissues, and organ functions, which aids in establishing and progressing Kimura disease.

• The frequent co-occurrence with atopic dermatitis (dry and itchy skin), and bronchial asthma, and drug allergies suggests that the cause is immune-dependent.

What Are the Symptoms of Kimuras Disease?

Symptoms of Kimura’s disease are as follows:

• The soft tissues of the neck, head (parotid region, eye orbit, eyelid), inguinal region, and armpits are the most frequently affected by Kimura's disease.

• In the superficial and deep layers of the skin, tumors can take the shape of dense, spherical papules with clear borders that range in size from 1 to 20 millimeters or nodes that can measure up to seven centimeters in diameter.

• Pimples have a smooth, pinkish-purple appearance, are prone to bleeding, and occasionally have little crusts on top of them.

• The nodes do not bleed, but they have a brownish tinge. A small residual pigmentation may remain after resolution.

• Local salivary glands and lymph nodes become involved over time. Nothing alters the skin above them.

• Lymph nodes with a thick, elastic substance are movable and painless to touch.

• The mucous membranes have occasionally developed hemorrhagic lesions.

• The general state of health is unaffected. Internal organs are unaffected.

• The patient's appearance is unintentionally altered by the slow growth of solitary or many nodes, which is an aesthetic issue.

How Is the Disease Diagnosed?

There are currently no standardized diagnostic standards.

• The diagnosis of Kimura's illness may be tentatively made based on the clinical signs and the results of blood tests, but a pathological examination is necessary for a firm determination.

• Kimura disease's histological features include follicular hyperplasia, eosinophilic infiltrates, and increased postcapillary venules.

• It may be possible to identify the tumor's features, borders, and blood supply and the presence of lymph node involvement using physical examination in conjunction with ultrasonography, CT (computed tomography), and MRI (magnetic resonance imaging).

• The mass could appear heterogeneous or homogeneous on ultrasonography, occasionally showing enhanced vascularity. Ultrasonography uses high-frequency sound waves to create black-and-white images to study the body's internal organs and tissues.

• The lesions are strongly enhanced on computed tomography, demonstrating their vascular origin. Lymphadenopathy is listed as one of the common findings. By computer processing, a computed tomography (CT) scan can produce cross-sectional images (slices) of the bones, blood arteries, and soft tissues inside the body by combining several X-ray images collected from various angles all over the body.

• On MRI, the lesion displays hyperintense signals on T2-weighted pictures and intermediate to high signal intensities on T1-weighted images. A powerful magnetic field and radio waves are used in an MRI scan to produce images on a computer screen. It can display inside organs, tissues, and other bodily structures.

• Imaging is a useful tool to illustrate the form and size of the lesion as well as its relationship with nearby structures.

What Are the Differential Diagnoses?

Some differential diagnoses are as follows:

• Sarcoidosis: The development of tiny clusters of inflammatory cells in any body area, most frequently the lymph nodes and lungs.

• Lymphoma: A lymphatic system cancer, a component of the body's defense against infection. The spleen, thymus gland, lymph nodes (lymph glands), and bone marrow are all components of the lymphatic system.

• Kaposi's Sarcoma: A form of cancer that develops in the lining of blood and lymph vessels. Kaposi's sarcoma tumors (lesions) often present as painless purplish spots on the legs, foot, or face. Additionally, lesions may develop in the mouth, lymph nodes, or the genital area.

• Vascular Tumors: A blood vessel or lymph vessel-based soft tissue growth that is benign or cancerous.

• Mikulich's Disease: The diffuse and bilateral swelling of the salivary and lacrimal glands.

How Is Kimuras Disease Treated?

Kimura's illness does not have a well-established treatment standard. At this time, treatment aims to preserve look and functionality while avoiding recurrence and long-term consequences such as nephritis and myocarditis.

Observation is acceptable if the lesions from Kimura disease are neither symptomatic nor disfiguring.

• Corticosteroids: Oral corticosteroids are widely utilized; however, if therapy is stopped, the condition frequently returns. To minimize associated nodular masses, oral corticosteroids combined with Cetirizine may be a successful alternative to surgery.

• Leflunomide: Patients refractory to corticosteroids alone may be treated with Leflunomide in conjunction with oral Prednisolone. On eosinophils, Leflunomide may have an antiproliferative impact. After receiving treatment with Leflunomide and Methylprednisolone, one study's patient was still free of disease at the 12-month checkup.

• Anti-IgE Medication: It was recently launched. It has been shown that patients with Kimura disease who received anti-IgE therapy experienced a decrease in lesion size and peripheral blood eosinophil count.

• Surgical Resection: According to the broadest retrospective meta-analysis of Kimura disease treatment, the least likely recurrence method was surgical resection with low-dose postoperative radiation. Additionally, it recommended that corticosteroid medication be utilized as a second-line treatment option due to the high rates of recurrence when used alone and the probable side effects of long-term corticosteroid use.

What Is the Prognosis of the Disease?

• Lesions typically persist or recur in the primary site despite therapy in Kimura disease, with a recurrence incidence of up to 25 %. Kimura’s disease presents a significant challenge to the treating physician and the patient.

• Kimura disease, which affects the head and neck, can cause a deformity due to the progression of untreated lesions. Recurrence following treatment is also adequately described.

• The disease's poor prognosis has also been linked to smoking behaviors and a history of systemic disease.

• The literature still needs to include descriptions of the malignant transformation of Kimura disease.

Conclusion:

Kimura’s disease is a rare long-standing disorder characterized by painless swelling below the skin in the head and neck regions. A histological examination of the sample diagnoses it. Consult a physician whenever any symptoms appear that resemble the disease for the appropriate treatment.