Kimura disease
Description
● Introduction:
➣ Originally identified as "eosinophilic hyperplastic lymphogranuloma" in 1937, Kimura disease (KD) was formally recognised as Kimura's disease in 1948. With a male-to-female ratio of 3:1, it is uncommon outside of Asia and most commonly observed in adolescent Asian men. There have only been about 200 instances recorded globally since its histological description.
➣ Although salivary glands and lymph nodes may be affected, the illness usually affects the head and neck area (periauricular, submandibular, orbit, and eyelids). 10–60% renal involvement is a common systemic connection. Histopathology is used for diagnosis since cytological findings are sometimes vague.
● Discussion:
➣ Epidemiology: Can happen at any age, but primarily affects young Asian males aged 20 to 40.
➣ Etiopathogenesis: Supposed to be an autoimmune or allergic reaction to continuous antigenic stimulation. Cytokine overproduction (IL-4, IL-5, TNF-α, GM-CSF, eotaxin, RANTES), elevated IgE levels, and eosinophilia are caused by an overactive Th2 (CD4+) T-cell response. Clinical course: benign, slow, and chronic; sporadic spontaneous regression has been noted.
➣ Systemic association: Nephrotic syndrome and renal illness, particularly membranous glomerulonephritis.
● Histological characteristics (Hui et al. classification):
➣ Constant: Postcapillary venule growth, florid germinal centre hyperplasia, eosinophilic infiltration, and preserved nodal architecture Fibrosis, proteinaceous deposits, reticular IgE deposition, and eosinophilic abscesses are common.
➣ Progressive change of the germinal centre is uncommon.
● Differential Diagnosis:
➣ Angiolymphoid hyperplasia with eosinophilia (ALHE): Main mimic; identified by vascular lobules with atypical epithelioid endothelial cells, female preponderance, lack of eosinophilia/IgE increase, and superficial dermal papulonodules.
➣ Other conditions include drug-induced lymphadenitis, dermatopathic lymphadenopathy, parasite lymphadenitis, Kaposi sarcoma, Hodgkin lymphoma, and Castleman disease.
Report on the Case:
➣ Male patient, age 14
➣ Presentation: No constitutional symptoms, two years of painless, increasing left submandibular swelling Examination: non-adherent to mandible, 5×2 cm lobulated, firm, non-tender swelling, normal skin overlaying it. Research:
The TLC is 9,500/cumm (neutrophils 32%, lymphocytes 42%, eosinophils 24%, and monocytes 2%). The Hb is 12.5 g/dl.
• Sufficient platelets
➣ FNA results: haemorrhage, fibrous stroma, endothelial clusters, polymorphous lymphoid cells, histiocytes, multiple eosinophils, and chronic nonspecific lymphadenitis with eosinophilia.
➣ The results of the excision biopsy include:
• Endothelial proliferation in the medulla;
• Histiocytic and eosinophilic infiltration;
• Eosinophilic abscesses and proteinaceous deposits;
• Partial nodal effacement;
• Hyperplastic lymphoid follicles with germinal centres
➣ Kimura's illness is the diagnosis.
Protocol
● Management:
➣ Systemic corticosteroids: Effective but relapses after withdrawal;
➣ Surgical excision: Preferred for localised disease, but recurrence is common. For recurring or steroid-resistant lesions, radiotherapy is an option.
➣ Other treatments being tested include imatinib, leflunomide, and cyclosporine.
● Fundamental Nursing Issues in Kimura illness:
→Patients with Kimura illness face a number of social, psychological, and physical difficulties.
➣ Pain/Discomfort: Although swelling is "painless," its magnitude or anatomical obstruction may produce local discomfort.
➣Changed Body Image: Swelling in the face or neck might impact one's look and self-worth.
Infection Risk: Biopsies and swelling raise the risk of infection.
➣ Nutritional Compromise: Eating may be hampered by submandibular or facial oedema.
➣Emotional/Psychological Distress: Concern for chronic illness, diagnosis, and therapy.
➣ Knowledge Deficit: Patients and their families might not be aware of the nature, chronicity, or treatment of the condition.
● Comprehensive Nursing Care
➣ Comfort & Symptom Management
· Track and Evaluate Swelling
To quickly identify problems like infection or ulceration, regularly monitor and record changes in the swelling's size, colour, and discomfort.
• Pain Control
Even though it is usually painless, check for discomfort and administer painkillers if needed. supportive comfort measures (soft handling, cold compress if authorised).
➣ Prevention of Infections and Wound Care
Maintain sterile dressing technique if a biopsy or surgery is performed, and inform patients about infection symptoms including fever, increased redness, or discharge.
• Sterile Methods
Follow up on wound healing and make that any invasive operations and sample collection are carried out in a sterile environment.
➣ Nutritional Assistance
• Evaluation and Adjustment of Diet
Determine whether swelling makes it difficult to chew or swallow. If necessary, serve liquid or soft meals, and keep a close eye on dietary intake.
•Keep an eye on your weight and hydration.
Keep track of your weight and fluid intake to make sure you're getting enough nourishment as you heal.
➣ Psychosocial Support
• Body Image Counseling
To address concerns about swelling in the face or neck and changes in appearance, provide supportive counselling.
Reduction of Anxiety
To lessen worry, reassure, thoroughly explain procedures and treatments, and welcome enquiries.
• Education for Patients and Families
Provide comprehensive information regarding Kimura illness, including its benign course, recurrence risk, available treatments (such as steroids, surgery, or observation), and the necessity of routine follow-up.
➣ Keep an eye out for complications
• Regular Follow-Up
Plan follow-up appointments to keep an eye on recurrence, new symptoms, treatment side effects, or the emergence of complications (particularly if immunosuppressive medication is being used).
• Laboratory Surveillance
Monitor IgE and eosinophil counts to gauge therapy response and disease activity.
Notes
For more details visit 10.15386/cjmed-1030
