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Case 198 - Discussion

Hematopathology Case

 

Kimura Disease

The Key Features

  • Young Asian, head and neck.

  • Prominent eosinophilia, follicular hyperplasia and vascularization, postcapillary venule proliferation, eosinophilic abscess and eosinophilic folliculolysis.

  • Elevated serum IgE and peripheral blood eosinophilia

GENERAL FEATURES

  • Also named eosinophilic hyperplastic lymphogranuloma, eosinophilic lymphofolliculosis and eosinophilic lymphofollicular granuloma.

  • A chronic inflammatory disorder of unknown etiology.

  • Location: skin, lymph nodes, salivary gland, and subcutaneous in the head and neck region.

  • Clinical: painless; often elevated serum IgE and peripheral blood eosinophilia.

  • Most cases have been reported in Asians; male predominant, M:F = 6:1; median age of 28 years.

GROSS FINDINGS

  • Solitary enlarged painless lymph node or generalized lymphadenopathy. Frequent salivary gland involvement.

  • Single or multiple subcutaneous nodules usually located on the head or neck, especially in the periauricular, parotid, or submandibular regions.

  • Involvement of the extremities and inguinal lymph nodes has been reported.

  • Average diameter of lesions, 3 cm.

MICROSCOPIC FINDINGS

  • Preserved nodal architecture, florid germinal center hyperplasia and vascularization, eosinophilic infiltration, and postcapillary venule proliferation.

  • Necrosis of the germinal centers, eosinophilic abscess, eosinophilic folliculolysis, polykaryocytes, proteinaceous deposits in the germinal centers, stromal and perivenular sclerosis, and a reticular IgE deposition within germinal centers.

DIFFERENTIAL DIAGNOSES

  • Angiolymphoid hyperplasia with eosinophilia (ALHE, epithelioid hemangioma)

    • ALHE is a neoplasm of blood vessels with arteriovenous malformation and secondary inflammation, whereas Kimura disease is a primary chronic inflammatory disorder with secondary vascular proliferation.

    • Commonly seen in Western individuals in the third to fifth decade in all racial groups with a slight female predominance,

    • Regional lymphadenopathy, serum eosinophilia, and elevated IgE levels are uncommon in ALHE.

    • Patients present with small, superficial dermal papulonodules, frequently erythematous, accompanied by bleeding and pruritis.

    • Microscopically, Kimura has three components: cellular proliferation: prominent eosinophilia and follicular hyperplasia.; fibrocollagenous; vascular hyperplasia: arborizing vascular proliferation of the postcapillary venule. By comparison, the vascular proliferation is most significant in ALHE, forming aggregates or lobules comprised of plump endothelial cells with epithelioid or histiocytoid changes and frequently demonstrating cytologic atypia and vacuolization.

TREATMENT AND PROGNOSIS

  • The disease has an excellent prognosis with no potential for malignant transformation.

  • Surgery is the mainstay of therapy, although regional or systemic corticosteroid therapy, cytotoxic therapy, and radiation have been used.

  • Recurrence after surgery or discontinued steroid treatment is common.

REFERENCES

  • Kimura disease: Diagnosis and prognostic factors. Otolaryngology–Head and Neck Surgery (2007) 137, 306-311

  • http://www.emedicine.com/derm/topic212.htm

  • Arch Pathol Lab Med. 2007;131:650–651)

  • Am J Surg Pathol 2004;28:505–513)