Kimura's disease
(Redirected from Eosinophilic lymphofollicular granuloma)
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Kimura's disease | |
---|---|
Synonyms | Eosinophilic hyperplastic lymphogranuloma |
Pronounce | N/A |
Specialty | N/A |
Symptoms | Painless subcutaneous nodules, lymphadenopathy, eosinophilia |
Complications | Renal disease |
Onset | Typically in young adult males |
Duration | Chronic |
Types | N/A |
Causes | Unknown |
Risks | Asian descent, male gender |
Diagnosis | Biopsy, blood tests showing eosinophilia |
Differential diagnosis | Angiolymphoid hyperplasia with eosinophilia, lymphoma, salivary gland tumors |
Prevention | N/A |
Treatment | Corticosteroids, surgical excision, radiation therapy |
Medication | N/A |
Prognosis | Generally good, but may recur |
Frequency | Rare |
Deaths | N/A |
Kimura's disease is a rare, chronic inflammatory disorder that primarily affects the head and neck regions. It is characterized by the presence of painless subcutaneous nodules, lymphadenopathy, and elevated levels of eosinophils in the blood. The disease is most commonly observed in young adult males of Asian descent.
Clinical Presentation
Patients with Kimura's disease typically present with painless, slow-growing masses in the head and neck regions, particularly in the parotid gland and submandibular gland. These masses are often accompanied by lymphadenopathy and may be associated with pruritus and erythema. In some cases, patients may also exhibit nephrotic syndrome due to renal involvement.
Pathophysiology
The exact cause of Kimura's disease remains unknown. However, it is believed to be an immune-mediated disorder. Histologically, the disease is characterized by the presence of lymphoid follicles, eosinophilic infiltration, and vascular proliferation. The elevated levels of eosinophils and IgE suggest an underlying allergic or hypersensitivity reaction.
Diagnosis
The diagnosis of Kimura's disease is primarily based on clinical presentation and histopathological findings. Imaging studies such as ultrasound, CT scan, and MRI can help in assessing the extent of the disease. A definitive diagnosis is made through a biopsy of the affected tissue, which reveals characteristic histological features.
Treatment
There is no definitive cure for Kimura's disease. Treatment is primarily aimed at managing symptoms and may include corticosteroids, immunosuppressive drugs, and surgical excision of the nodules. In cases of renal involvement, treatment may also involve managing nephrotic syndrome.
Prognosis
Kimura's disease is a benign condition with a good prognosis. However, it is a chronic disease with a tendency for recurrence. Long-term follow-up is recommended to monitor for potential complications and recurrences.
See also
- Lymphadenopathy
- Eosinophilia
- Parotid gland
- Submandibular gland
- Nephrotic syndrome
- Immune-mediated disorder
References
External Links
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Contributors: Prab R. Tumpati, MD