Lymph nodes & spleen, nonlymphoma

Lymph nodes-inflammatory / reactive disorders

Kikuchi disease



Last author update: 15 February 2022
Last staff update: 15 February 2022

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PubMed Search: Kikuchi disease[TI]

Amy Beckman, M.D.
Elizabeth Courville, M.D.
Page views in 2021: 24,315
Page views in 2022 to date: 26,812
Cite this page: Beckman A, Courville E. Kikuchi disease. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lymphnodeskikuchi.html. Accessed December 3rd, 2022.
Definition / general
  • Benign, self limited lymphadenitis of unknown etiology with distinct histopathologic features
Essential features
  • Benign, self limited lymphadenitis of unknown etiology
  • Predominantly affects young adults, with cervical lymphadenopathy, fever and often leukopenia
  • Involved lymph nodes show histiocytes, plasmacytoid dendritic cells, immunoblasts and necrosis with karyorrhectic debris
  • Histologic features overlap with autoimmune disease associated lymphadenitis, especially systemic lupus erythematosus
  • Clinically and histologically may mimic lymphoma
Terminology
  • Kikuchi-Fujimoto disease
  • Kikuchi lymphadenitis
  • Histiocytic necrotizing lymphadenitis
  • Subacute necrotizing lymphadenitis
ICD coding
  • ICD-10: I88 - nonspecific lymphadenitis
Epidemiology
Sites
  • Cervical lymph nodes, especially posterior
  • Other lymph nodes less commonly involved (axillary, mediastinal, abdominal, inguinal)
  • Rarely, extranodal sites, including skin
Pathophysiology
Etiology
Clinical features
Diagnosis
  • Lymph node excision with pathologic examination
Laboratory
Case reports
Treatment
Microscopic (histologic) description
  • Partial or complete lymph node involvement by irregularly shaped, pale areas composed of histiocytes, plasmacytoid dendritic cells, eosinophilic granular material and abundant karyorrhectic debris (nuclear dust), often surrounding a central zone of overt necrosis
  • Karyorrhectic areas may extend beyond nodal capsule or involve residual germinal centers
  • Regions between pale areas include small lymphocytes admixed with immunoblasts and clusters of plasmacytoid dendritic cells, causing a mottled or starry sky appearance
  • Histiocytes include phagocytic cells with eosinophilic cytoplasm and crescent shaped nuclei (crescentic histiocytes) and nonphagocytic cells with twisted or reniform nuclei (Am J Surg Pathol 1994;18:219)
  • Plasmacytoid dendritic cells are intermediate in size with amphophilic cytoplasm, round nuclei, small nucleoli; difficult to recognize on H&E (Diagn Cytopathol 2010;38:521)
  • No neutrophils; rare to absent plasma cells (Clin Rheumatol 2007;26:50)
  • Overt necrosis not required for diagnosis (Am J Surg Pathol 1994;18:219)
  • 3 stages described based on histology (Am J Surg Pathol 1995;19:798, Pathol Int 2004;54:237):
    • Proliferative: proliferation of plasmacytoid dendritic cells, immunoblasts and histiocytes
    • Necrotizing: areas of necrosis with karyorrhectic debris
    • Xanthomatous: foamy histiocytes predominate; may represent histologic variant
Microscopic (histologic) images

Contributed by Elizabeth Courville, M.D. and Amy Beckman, M.D.

Lymph node from a 38 year old woman:
Distorted lymph node architecture

Distorted lymph node architecture

Pale staining areas

Pale staining areas

Karyorrhectic debris

Karyorrhectic debris

Histiocytes and lymphocytes

Histiocytes and lymphocytes


CD3, CD4, CD8, CD20

CD3, CD4, CD8, CD20

CD3, CD4, CD8, CD68

CD3, CD4, CD8, CD68

CD30, CD123, MPO, EBV ISH

CD30, CD123, MPO, EBV ISH



Cervical lymph node from a 40 year old woman:
Pale staining areas

Pale staining areas

Necrosis with mononuclear infiltrate

Necrosis with mononuclear infiltrate

Karyorrhectic debris

Karyorrhectic debris

Histiocytes

Histiocytes

Lymph node

Lymph node


CD68

CD68

CD123

CD123

CD3

CD3

CD20

CD20

CD30

CD30



AFIP images

Kikuchi necrotizing lymphadenitis

Virtual slides

Images hosted on other servers:

Lymph node excision

Needle core biopsy

CD3 stain

CD30 stain

CD68 stain

CD123 stain

Cytology description
  • Phagocytic histiocytes with peripheral nuclei; plasmacytoid dendritic cells
Positive stains
Electron microscopy description
Electron microscopy images

Images hosted on other servers:

Histiocytes with myelin figures

#2 with tubuloreticular structures

Sample pathology report
  • Lymph node, cervical, excision:
    • Necrotizing lymphadenitis (see comment)
    • Comment: The findings favor a diagnosis of Kikuchi disease, a benign and typically self limited lymphadenitis. There is no evidence of lymphoma. Histologically, changes associated with autoimmune disease may mimic those seen in Kikuchi disease. Clinical correlation, along with evaluation for a possible autoimmune disease, such as systemic lupus erythematosus, is recommended.
    • Microscopic description: H&E stained sections show a lymph node with architecture distorted by pale areas composed of eosinophilic, granular material, karyorrhectic debris and interspersed lymphocytes and histiocytes. These areas are surrounded by histiocytic cells (some with twisted nuclei and others with so called crescentic nuclei) and cells with morphology suggestive of plasmacytoid dendritic cells. Outside the pale areas, there is an expansion of small lymphocytes admixed with immunoblasts. Residual primary and secondary follicles are present but neutrophils are not seen and plasma cells are rare to absent. Neither hematoxylin bodies nor aggregates of large atypical lymphocytes are identified. Immunohistochemical stains are performed with appropriate controls. CD3 highlights numerous T cells; CD8 positive cells outnumber CD4 positive cells. CD20 stains residual nodules of B cells. CD68 stains histiocytes within and adjacent to karyorrhectic areas. CD123 highlights plasmacytoid dendritic cells, which surround and form clusters outside of karyorrhectic areas. CD4 shows weak staining in both histiocytes and plasmacytoid dendritic cells.
Differential diagnosis
  • Lupus lymphadenitis:
    • Hematoxylin bodies, Azzopardi phenomenon, plasma cells
    • CD4+ T cells > CD8+ T cells
    • Some cases may be histologically indistinguishable from Kikuchi disease
  • Infectious lymphadenitis:
    • Neutrophils, granulomatous inflammation, viral cytopathic changes
  • Lymphoma (e.g., diffuse large B cell lymphoma, peripheral T cell lymphoma, NOS, Hodgkin lymphoma):
    • Nodal effacement by monotonous or atypical lymphocytes or presence of Reed-Sternberg / Hodgkin cells
    • Pattern of staining seen with a panel of immunohistochemical stains (for example, CD3, CD20, CD30, PAX5) and interpreted in the context of morphology on routine hematoxylin and eosin stain is important in making the distinction from Kikuchi disease
Board review style question #1
Which disease shows similar morphologic and immunohistochemical features to Kikuchi lymphadenitis?

  1. Classic Hodgkin lymphoma
  2. Rheumatoid arthritis
  3. Systemic lupus erythematosus
  4. Toxoplasmosis
Board review style answer #1
C. Systemic lupus erythematosus

Comment Here

Reference: Kikuchi disease
Board review style question #2

In this lymph node involved by Kikuchi disease, a proliferation of which cell type is illustrated by immunohistochemical stain for CD123?

  1. Crescentic histiocytes
  2. Immunoblasts
  3. Monocytoid B cells
  4. Plasmacytoid dendritic cells
Board review style answer #2
D. Plasmacytoid dendritic cells

Comment Here

Reference: Kikuchi disease
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