Age / Sex : 16 / M

Chief complaint :

1. Waxing and waning palpable masses, medial elbow, Lt. (onset: 2 YA)


2. Proptosis, Rt. (onset: 2 YA)

• Fever (-), Chill (-), Dyspnea (-)


• TFT: normal, IgG4: normal, CBC: eosinophilia

Kimura’s disease (proven by surgical excision of an epitrochlear lymph node)

Discussion

Findings:

 Epitrochlear lymphadenopathy and unilateral swelling of extraocular rectus muscles, with peripheral blood eosinophilia

Differential Diagnosis:

 Inflammatory pseudotumor, other lymphoproliferative diseases…

Discussion:

• Kimura’s disease is a benign, rare, chronic inflammatory condition with several proposed etiologies including an aberrant immune reaction, immunoglobulin E-mediated hypersensitivity reaction, trauma, or chronic infection.


• Most cases have peripheral blood eosinophilia and elevated serum IgE levels.


• Classically presents in young Asian men.


• A painless, slow-growing, subcutaneous nodule usually manifesting on the face or neck with the parotid region being the most common site of involvement. Orbital involvement is rare, but when occurs usually affects the lacrimal glands or eyelids. Regional or satellite lymphadenopathy may be present. Unilateral swelling of a rectus muscle may be the first sign of Kimura’s disease of the orbit prior to the development of proptosis, while lymphadenopathy may be the first presenting sign in systemic disease.


• The final diagnosis relies on typical histopathology findings of the mass or lymph node.


• Due to its rarity, no standard treatment has been established; however, efficacy has been reported in small case series and individual case reports in which the preferred treatment for localized disease is surgery. Surgical excision with post-operative radiation has shown the smallest risk of recurrence; however, local radiation therapy alone, systemic corticosteroid and immunosuppressant therapy, leukotriene receptor antagonists, and histamine receptor blockers have all shown success.

References:

 http://www.ajnr.org/ajnr-case-collections-diagnosis/kimura’s-disease

 Am J Surg Pathol 2004;28:505-513