Kimura disease (Angio-lymphoid hyperplasia with eosinophilia, ALHE)

Definition

Kimura disease is a chronic, benign, inflammatory disease of unknown, possible allergic cause with typical swelling under the skin and swollen lymph nodes on the head or neck. Eosinophilic white blood cells leukocytes and IgE most often elevated in blood tests. The disease was first described in China in 1936 (by Kimura). Angio-lymphoid hyperplasia with eosinophilia (ALHE) is believed to be the same condition.

• Kimura disease can sometimes remind you of some rheumatic diseases.

Occurrence

Rarely described outside Asia.

• Young men are affected 6 times more frequently than women. Median debut age 28 year
• Kimura disease (KFS) is defined as one rare disease

Symptoms

Skin, lymph nodes and salivary glands are attacked. Slowly growing painless nodes under the skin are typical. Can itch. Renal failure with nephrotic syndrome has also been reported.

• Subcutaneous swelling
• Swollen lymph nodes on the head or neck
• Eosinophilic leukocytes og IgE are most often elevated in the blood

Medical examinations

At clinical examination detected enlarged, non-painful lymph nodes and often enlarged salivary glands. One or more nodules under the skin of the head or neck, especially in the ears, parotid or submandibular areas. Less often on eyelids. The average diameter is 3 cm. Changes in arms and legs have also been reported.

Laboratory tests

• White blood cells with differential count usually show eosinophilia (98%) in blood samples. IgE is often elevated. Creatinine may be elevated if the kidneys are attacked
• Urin test

CT or MRI examinations

• Image diagnostics can show the spread of the disease

Biopsy (Tissue Test)

• Tissue sample should be taken to confirm the diagnosis. Eosinophilic infiltrates and eosinophilic abscesses are typical. Fibrosis may be peripheral to the infiltrates

Incorrect diagnosis? (similar diseases / differential diagnoses

• Angiolymphoid Hyperplasia with eosinophilia (probably the same condition, reference: Ramaprasad AD 1993)
• Cylindrome
• Dermatofibrosarcoma Protuberans
• Eccrine cylinder
• Eosinophilic granuloma
• Kaposi Sarcom
• Lymph node metastases
• Lymphoma
• Mikulicz disease
• Pyogenic Granuloma (Lobular Capillary Hemangioma)
• Reactive lymphadenopathy (swollen lymph nodes by infection or rheumatic inflammation)
• Sjögren's syndrome
• Salivary Gland Tumor

Treatment

Observation. Steroid injections as topical treatment can reduce the infiltrates. Cetrizine reduces itching. Systemic corticosteroids may be appropriate. Cyclosporine is used in some cases. Similarly for azathioprine (Imurel). Surgical treatment can be done, but recurrence is common.

Literature

• Chitrapu C, 2014 (Angio-lymphoid hyperplasia with eosinophilia)
• Wikipedia (English)

---

• Bindevevssykdommer.no
• Vaskulitt.no