Kimura Disease

Kimura Disease

C. Cameron Yin, MD, PhD

Low-power view shows a lymph node and perinodal soft tissue involved by Kimura disease. This field reveals hyperplastic lymphoid follicles image, marked eosinophilia, and fibrosis.

A lymph node involved by Kimura disease is pictured with marked replacement of the interfollicular regions by lymphocytes and eosinophils. A reactive follicle is present image.



  • Kimura lymphadenopathy

  • Eosinophilic lymphogranuloma

  • Eosinophilic lymphoid follicular hyperplasia


Infectious Agents

  • History and histologic findings suggest infectious etiology

    • No definite pathogen has been identified

Other Proposed Causes

  • Allergy

  • Autoimmunity



  • Age

    • Mainly occurs in young adults

    • Peak age of onset in 2nd and 3rd decades

  • Gender

    • Predominantly males

  • Ethnicity

    • Asians are most commonly afflicted

    • Named after T. Kimura from Japan who reported a case in 1948


  • Usually in head & neck region

  • Involves deep subcutaneous tissues

    • Regional lymph nodes

  • Often involves major salivary glands

    • Parotid, submandibular


  • Nontender masses in head & neck

    • Most often in periauricular region

  • Rarely patients have generalized lymphadenopathy

  • Systemic symptoms are uncommon

Laboratory Tests

  • Peripheral blood eosinophilia almost invariable

  • Elevated serum IgE level

  • Elevated erythrocyte sedimentation rate (ESR)

  • Imbalance between Th1 and Th2 cytokines

    • Increased TNF-α, IL-4, IL-5, IL-13, etc.

Natural History

  • Insidious onset

  • Slow-growing mass

    • Interval from onset of swelling to presentation may be several years

    • Often persists unchanged for years

  • Nephrotic syndrome may occur in up to 60% of patients

  • Some may have allergic conditions such as urticaria or eczema


  • Indolent clinical course

  • Recurrence after excision and steroid therapy is common


General Features

  • Ultrasound, CT, or MR scans are useful for determining extent of disease

  • Combination of ultrasonography and MR has been shown to have high diagnostic value

    • Hypoechoic center and hyperechoic margin with enriched blood vessels on ultrasonography and Doppler

    • Hypointensity replaces normal hyperintense subcutaneous fat on MR

    • Lymph nodes are enlarged with well-defined outline

  • CT scan shows nonspecific findings


Histologic Features

  • Lymphoid infiltrate in deep subcutis

    • Formation of follicles with germinal centers

    • Accompanied by many eosinophils, plasma cells, and mast cells

      • Eosinophilic microabscesses can be present

    • Vascular hyperplasia

  • Lymph nodes show preserved but distorted overall architecture with

    • Hyperplastic follicles with well-formed germinal centers and mantle zones

    • Deposition of IgE in germinal centers forms hyaline proteinaceous material

    • Eosinophilia

      • Eosinophilic microabscesses and eosinophilic follicle lysis

      • Involvement of perinodal soft tissues

    • Necrosis (±); usually not extensive

    • Vascular proliferation in interfollicular regions

      • Endothelial cells lack cuboidal/polygonal shape, e.g., “hobnail” or “tombstone” appearance (seen in ALHE)

    • Stromal and perivascular sclerosis

Cytologic Features

  • Fine-needle aspiration (FNA) yields polymorphous cell population with many eosinophils

    • Difficult to establish diagnosis of KD based on FNA findings alone



  • IgE deposits in the germinal centers can be shown by immunohistochemistry or immunofluorescence

  • Polytypic B cells and normal T cells

Molecular Genetics

  • No evidence of monoclonal gene rearrangements

  • No known translocations or oncogene abnormalities

  • No evidence of an infectious organism


Angiolymphoid Hyperplasia With Eosinophilia (ALHE)

  • ALHE has a number of other names

    • Epithelioid hemangioma is probably best name

      • Lesion is thought to be benign vascular neoplasm

    • Other names

    • Histiocytoid hemangioma

    • Pseudo- or atypical pyogenic granuloma

    • Inflammatory angiomatous nodule

    • Intravenous atypical vascular proliferation

  • Occurs more often in

    • Caucasians

    • Young to middle-aged adults

  • Presents as multiple papules or nodules

    • Usually occurs in head and neck region

    • Common around ear

  • Peripheral blood eosinophilia occurs in ˜ 15% of ALHE patients

  • Histologic findings of ALHE differ from Kimura disease as follows

    • Located in superficial dermis

    • Lesion has low-power lobular pattern of capillary or medium-sized blood vessels

    • Hypertrophic cuboidal/polygonal endothelial cells

      • Protrude into or occlude vascular lumina

      • Described as “hobnail” or “tombstone” appearance

    • Lesion can be located within large blood vessel

    • No lymph node involvement

  • Local recurrence can occur in ˜ 33% of patients

Langerhans Cell Histiocytosis

Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Kimura Disease

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