Kikuchi-Fujimoto disease (KFD), also called histiocytic necrotizing lymphadenitis (HNL), is a rare, benign, and typically self-limiting systemic disorder of obscure etiology, first described independently in Japan in 1972 by Kikuchi and Fujimoto. It most commonly affects young adults, historically with a female predominance and a higher reported incidence in Asian populations. The classic presentation is subacute, tender cervical lymphadenopathy with fever, often accompanied by night sweats, weight loss, leukopenia, and an elevated erythrocyte sedimentation rate, and sometimes skin rash, arthralgia, and hepatosplenomegaly. KFD is neither Mendelian nor neoplastic. Its diagnostic histopathologic hallmark is paracortical foci of necrosis with abundant karyorrhectic (nuclear) debris and a histiocytic infiltrate (crescentic histiocytes and plasmacytoid dendritic cells) together with CD8-positive cytotoxic T cells, with a characteristic absence of neutrophils and a paucity of plasma cells that helps distinguish it from lymphoma and lupus lymphadenitis. The mechanism is thought to be an exuberant, self-limited T-cell and histiocyte-mediated immune response in which apoptotic cell death is driven by CD8-positive cytotoxic T cells, possibly triggered by viral or other infectious agents in genetically predisposed individuals, though no causative agent has been proven. KFD has a strong recognized association with systemic lupus erythematosus and may precede, coincide with, or follow it, so lupus workup and long-term follow-up are important. The disorder usually resolves spontaneously within weeks to months; treatment is supportive (NSAIDs, analgesics), with corticosteroids and hydroxychloroquine reserved for severe or recurrent disease, and recurrence is uncommon.
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Conditions with similar clinical presentations that must be differentiated from Kikuchi-Fujimoto Disease:
name: Kikuchi-Fujimoto Disease
creation_date: "2026-06-30T00:00:00Z"
category: Complex
disease_term:
preferred_term: Kikuchi-Fujimoto disease
term:
id: MONDO:0018864
label: Kikuchi-Fujimoto disease
parents:
- Lymphadenitis
description: >-
Kikuchi-Fujimoto disease (KFD), also called histiocytic necrotizing
lymphadenitis (HNL), is a rare, benign, and typically self-limiting systemic
disorder of obscure etiology, first described independently in Japan in 1972
by Kikuchi and Fujimoto. It most commonly affects young adults, historically
with a female predominance and a higher reported incidence in Asian
populations. The classic presentation is subacute, tender cervical
lymphadenopathy with fever, often accompanied by night sweats, weight loss,
leukopenia, and an elevated erythrocyte sedimentation rate, and sometimes
skin rash, arthralgia, and hepatosplenomegaly. KFD is neither Mendelian nor
neoplastic. Its diagnostic histopathologic hallmark is paracortical foci of
necrosis with abundant karyorrhectic (nuclear) debris and a histiocytic
infiltrate (crescentic histiocytes and plasmacytoid dendritic cells) together
with CD8-positive cytotoxic T cells, with a characteristic absence of
neutrophils and a paucity of plasma cells that helps distinguish it from
lymphoma and lupus lymphadenitis. The mechanism is thought to be an
exuberant, self-limited T-cell and histiocyte-mediated immune response in
which apoptotic cell death is driven by CD8-positive cytotoxic T cells,
possibly triggered by viral or other infectious agents in genetically
predisposed individuals, though no causative agent has been proven. KFD has a
strong recognized association with systemic lupus erythematosus and may
precede, coincide with, or follow it, so lupus workup and long-term
follow-up are important. The disorder usually resolves spontaneously within
weeks to months; treatment is supportive (NSAIDs, analgesics), with
corticosteroids and hydroxychloroquine reserved for severe or recurrent
disease, and recurrence is uncommon.
references:
- reference: PMID:37383134
title: "Kikuchi-Fujimoto disease: A comprehensive review."
- reference: PMID:39961627
title: "Kikuchi-Fujimoto disease: investigating comprehensive clinicopathological features and risk factors for recurrence."
mechanistic_hypotheses:
- hypothesis_group_id: immune_dysregulation
hypothesis_label: Exuberant CD8 T-cell and interferon-driven immune response
status: CANONICAL
description: >-
KFD is modeled as an exaggerated but self-limited cell-mediated immune
response in which CD8-positive cytotoxic T cells and histiocytes drive
apoptotic (karyorrhectic) necrosis, with a plasmacytoid dendritic cell and
type I interferon component, sitting on a mechanistic spectrum toward
systemic lupus erythematosus. This is the best-supported framework, but the
precise trigger and initiating antigen remain unproven.
evidence:
- reference: PMID:9839173
reference_title: "Perforin and Fas pathways of cytotoxic T-cells in histiocytic necrotizing lymphadenitis."
supports: SUPPORT
evidence_source: IN_VITRO
snippet: >-
In histiocytic necrotizing lymphadenitis (HNL), apoptosis is the main
form of cell death.
explanation: >-
Immunohistochemical and flow-cytometric analysis of HNL nodal tissue
establishes apoptosis as the principal form of cell death, the basis of
the immune-dysregulation model.
- hypothesis_group_id: infectious_trigger
hypothesis_label: Hypothesized viral or infectious trigger
status: EMERGING
description: >-
A viral or other infectious agent is hypothesized to trigger KFD in a
genetically susceptible host, based on temporal, serological, and case
associations with agents such as EBV and human herpesviruses and on an
interferon signature. No organism has been consistently isolated or shown
to be causal, so the infectious etiology remains a hypothesis rather than
an established fact.
evidence:
- reference: PMID:24594231
reference_title: "Comparison of clinical features and EBV expression in histiocytic necrotizing lymphadenitis of children and adults."
supports: PARTIAL
evidence_source: HUMAN_CLINICAL
snippet: >-
Epstein-Barr virus (EBV) has been implicated as a cause of HNL in some
reports but not in others.
explanation: >-
Directly documents that a viral (EBV) etiology has been proposed but is
inconsistent across studies, supporting an unproven infectious-trigger
hypothesis rather than established causation.
- reference: PMID:36451640
reference_title: "Kikuchi-Fujimoto Disease: A Rare Cause of Pyrexia of Unknown Origin and Cervical Lymphadenopathy."
supports: PARTIAL
evidence_source: HUMAN_CLINICAL
snippet: >-
Although the clinical and histopathological features point to a viral
etiology, this hypothesis has not been proven yet.
explanation: >-
Explicitly frames the viral etiology as an unproven hypothesis,
supporting the emerging (not canonical) status of the infectious trigger.
pathophysiology:
- name: CD8 T-Cell- and Histiocyte-Mediated Apoptotic Necrosis
description: >-
In KFD lesions, an exaggerated cell-mediated immune response produces
apoptotic cell death driven predominantly by CD8-positive cytotoxic T
cells, acting through the Fas-Fas ligand and perforin/granzyme pathways.
Histiocytes (macrophages) act as enhancers and phagocytose the apoptotic
debris, generating paracortical foci of necrosis rich in karyorrhectic
(nuclear) debris characteristically without neutrophils.
cell_types:
- preferred_term: CD8-positive cytotoxic T cell
term:
id: CL:0000625
label: CD8-positive, alpha-beta T cell
- preferred_term: Histiocyte (macrophage)
term:
id: CL:0000235
label: macrophage
biological_processes:
- preferred_term: Apoptotic process
term:
id: GO:0006915
label: apoptotic process
modifier: INCREASED
- preferred_term: T cell mediated cytotoxicity
term:
id: GO:0001913
label: T cell mediated cytotoxicity
modifier: INCREASED
- preferred_term: Extrinsic (Fas) apoptotic signaling
term:
id: GO:0097191
label: extrinsic apoptotic signaling pathway
modifier: INCREASED
downstream:
- target: Paracortical Necrosis with Karyorrhexis and Self-Limited Lymphadenitis
description: >-
CD8 T-cell- and histiocyte-driven apoptosis produces the paracortical
karyorrhectic necrosis that manifests clinically as self-limited
lymphadenitis.
evidence:
- reference: PMID:9839173
reference_title: "Perforin and Fas pathways of cytotoxic T-cells in histiocytic necrotizing lymphadenitis."
supports: SUPPORT
evidence_source: IN_VITRO
snippet: >-
Two molecular mechanisms of T-cell-mediated cytotoxicity, one
perforin-based and the other Fas-based, have been demonstrated, and both
systems induce apoptosis of the target cells.
explanation: >-
Immunohistochemistry of HNL nodal tissue shows Fas/FasL- and
perforin/granzyme-positive cells confined to necrotizing lesions, with
CD8-positive lymphocytes as effector and target cells and histiocytes as
enhancers, supporting CD8 T-cell-driven apoptotic necrosis.
- reference: PMID:9839173
reference_title: "Perforin and Fas pathways of cytotoxic T-cells in histiocytic necrotizing lymphadenitis."
supports: SUPPORT
evidence_source: IN_VITRO
snippet: >-
the apoptotic cells were T-cells, especially CD8-positive cells rather
than CD4-positive cells
explanation: >-
Identifies CD8-positive T cells as the predominant apoptotic population,
supporting the CD8-cytotoxic-T-cell-centered mechanism.
- name: Plasmacytoid Dendritic Cell Infiltration and Type I Interferon Signature
description: >-
Lesional areas contain a characteristic infiltrate of immature dendritic
cells, including plasmacytoid dendritic cells (CD123/CD303-positive), that
strongly express the interferon-alpha-inducible protein MxA, indicating a
type I interferon (interferon-alpha) response that parallels the
interferon signature seen in systemic lupus erythematosus.
cell_types:
- preferred_term: Plasmacytoid dendritic cell
term:
id: CL:0000784
label: plasmacytoid dendritic cell
biological_processes:
- preferred_term: Type I interferon-mediated signaling pathway
term:
id: GO:0060337
label: type I interferon-mediated signaling pathway
modifier: INCREASED
- preferred_term: Inflammatory response
term:
id: GO:0006954
label: inflammatory response
modifier: INCREASED
evidence:
- reference: PMID:19141377
reference_title: "Histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto disease): lesional cells exhibit an immature dendritic cell phenotype."
supports: SUPPORT
evidence_source: IN_VITRO
snippet: >-
the morphologically distinctive mononuclear cells in lesional areas
consisted of 2 populations of immature DCs: myeloid DCs immunoreactive
for CD1c with coexpression of myeloid antigens CD13 and CD33 and
plasmacytoid DCs immunoreactive for CD303 and CD123
explanation: >-
Cryostat-section immunophenotyping of KFD lesions identifies plasmacytoid
and myeloid immature dendritic cells as the distinctive lesional
mononuclear cells.
- reference: PMID:19141377
reference_title: "Histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto disease): lesional cells exhibit an immature dendritic cell phenotype."
supports: SUPPORT
evidence_source: IN_VITRO
snippet: >-
These cells were CD68+, strongly expressed the IFN-alpha inducible
protein MxA, and were nonreactive for fascin, a mature DC marker.
explanation: >-
Strong MxA expression by the lesional dendritic cells demonstrates a type
I interferon (interferon-alpha) signature in KFD.
- name: Paracortical Necrosis with Karyorrhexis and Self-Limited Lymphadenitis
description: >-
The converging cytotoxic and histiocytic response produces the clinical
hallmark: subacute, often tender regional (typically cervical)
lymphadenopathy with fever, arising from paracortical necrosis rich in
apoptotic karyorrhectic debris. The process is self-limited and usually
resolves spontaneously over weeks to months.
evidence:
- reference: PMID:39961627
reference_title: "Kikuchi-Fujimoto disease: investigating comprehensive clinicopathological features and risk factors for recurrence."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Kikuchi-Fujimoto disease (KFD) is a rare disease that typically manifests
with fever and cervical lymphadenopathy.
explanation: >-
Confirms the characteristic clinical outcome of the pathophysiologic
cascade: fever with cervical lymphadenopathy.
- reference: PMID:28613580
reference_title: "Kikuchi-Fujimoto Disease."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Kikuchi-Fujimoto disease typically follows a self-limiting course lasting
several months
explanation: >-
Documents the self-limited, spontaneously resolving natural history of
the lymphadenitis.
phenotypes:
- category: Clinical
name: Cervical lymphadenopathy
description: >-
Subacute, often tender, regional cervical lymphadenopathy is the hallmark
and most consistent presentation of KFD.
phenotype_term:
preferred_term: Cervical lymphadenopathy
term:
id: HP:0025289
label: Cervical lymphadenopathy
temporality: SUBACUTE
evidence:
- reference: PMID:36451640
reference_title: "Kikuchi-Fujimoto Disease: A Rare Cause of Pyrexia of Unknown Origin and Cervical Lymphadenopathy."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Kikuchi-Fujimoto disease (KFD) is a benign disorder characterized by
regional cervical lymphadenopathy with tenderness.
explanation: >-
Directly documents tender regional cervical lymphadenopathy as the
characteristic presentation.
- category: Clinical
name: Fever
description: Low-grade to moderate intermittent fever is a common presenting feature.
phenotype_term:
preferred_term: Fever
term:
id: HP:0001945
label: Fever
evidence:
- reference: PMID:39961627
reference_title: "Kikuchi-Fujimoto disease: investigating comprehensive clinicopathological features and risk factors for recurrence."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Kikuchi-Fujimoto disease (KFD) is a rare disease that typically manifests
with fever and cervical lymphadenopathy.
explanation: Documents fever as a typical manifestation of KFD.
- category: Clinical
name: Night sweats
description: Night sweats are a common associated constitutional symptom.
phenotype_term:
preferred_term: Night sweats
term:
id: HP:0030166
label: Night sweats
evidence:
- reference: PMID:36451640
reference_title: "Kikuchi-Fujimoto Disease: A Rare Cause of Pyrexia of Unknown Origin and Cervical Lymphadenopathy."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Associated symptoms of KFD include low-grade fever, night sweats, weight
loss, nausea, and sore throat.
explanation: Lists night sweats among the associated constitutional symptoms of KFD.
- category: Clinical
name: Weight loss
description: Weight loss is an associated constitutional symptom, more common in severe disease.
phenotype_term:
preferred_term: Weight loss
term:
id: HP:0001824
label: Weight loss
evidence:
- reference: PMID:37383134
reference_title: "Kikuchi-Fujimoto disease: A comprehensive review."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
weight loss, splenomegaly, leucopenia, and elevated erythrocyte
sedimentation rate feature in severely affected patients
explanation: Documents weight loss as a feature of severely affected KFD patients.
- category: Laboratory
name: Leukopenia
description: >-
Leukopenia (decreased total leukocyte count) is the commonest laboratory
abnormality in KFD.
phenotype_term:
preferred_term: Leukopenia
term:
id: HP:0001882
label: Decreased total leukocyte count
evidence:
- reference: PMID:37383134
reference_title: "Kikuchi-Fujimoto disease: A comprehensive review."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
weight loss, splenomegaly, leucopenia, and elevated erythrocyte
sedimentation rate feature in severely affected patients
explanation: Documents leukopenia (leucopenia) as a laboratory feature of KFD.
- category: Laboratory
name: Elevated erythrocyte sedimentation rate
description: An elevated ESR is a common inflammatory laboratory finding in KFD.
phenotype_term:
preferred_term: Elevated erythrocyte sedimentation rate
term:
id: HP:0003565
label: Elevated erythrocyte sedimentation rate
evidence:
- reference: PMID:37383134
reference_title: "Kikuchi-Fujimoto disease: A comprehensive review."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
weight loss, splenomegaly, leucopenia, and elevated erythrocyte
sedimentation rate feature in severely affected patients
explanation: Documents an elevated erythrocyte sedimentation rate in KFD.
- category: Clinical
name: Skin rash
description: >-
Cutaneous involvement occurs in a substantial minority as facial erythema
and nonspecific erythematous papules, plaques, or acneiform/morbilliform
lesions.
phenotype_term:
preferred_term: Skin rash
term:
id: HP:0000988
label: Skin rash
frequency: FREQUENT
evidence:
- reference: PMID:37383134
reference_title: "Kikuchi-Fujimoto disease: A comprehensive review."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Cutaneous involvement occurs in about 30%-40% of cases as facial erythema
and nonspecific erythematous papules, plaques, acneiform or morbilliform
lesions
explanation: >-
Reports cutaneous involvement in about 30-40% of cases, supporting a
FREQUENT (30-79%) frequency band for skin rash.
- category: Clinical
name: Splenomegaly
description: Splenomegaly occurs in severely affected patients.
phenotype_term:
preferred_term: Splenomegaly
term:
id: HP:0001744
label: Splenomegaly
evidence:
- reference: PMID:37383134
reference_title: "Kikuchi-Fujimoto disease: A comprehensive review."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
weight loss, splenomegaly, leucopenia, and elevated erythrocyte
sedimentation rate feature in severely affected patients
explanation: Documents splenomegaly as a feature of severely affected KFD patients.
genetic:
- name: HLA-DPB1
notes: >-
KFD is not a Mendelian disorder and has no causal gene. The principal
genetic signal is an HLA class II susceptibility association: the
HLA-DPB1*0202 allele (and HLA-DPA1*01) is significantly over-represented in
Japanese KFD patients relative to controls. These are common germline
population variants conferring susceptibility, not pathogenic mutations,
and their higher frequency in East Asians may contribute to the observed
Asian predominance.
gene_term:
preferred_term: HLA-DPB1
term:
id: hgnc:4940
label: HLA-DPB1
variant_origin: GERMLINE
relationship_type: RISK_FACTOR
evidence:
- reference: PMID:10519361
reference_title: "DNA typing of HLA class II genes (HLA-DR, -DQ and -DP) in Japanese patients with histiocytic necrotizing lymphadenitis (Kikuchi's disease)."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
we found DPA1*01 and DPB1*0202 allele frequencies in HLA class II genes
are significantly higher in HNL patients than in normal controls
explanation: >-
A case-control DNA-typing study of 86 HNL patients versus 525 controls
found HLA-DPB1*0202 and HLA-DPA1*01 significantly over-represented,
establishing an HLA class II susceptibility association.
- name: HLA-DPA1
notes: >-
The HLA-DPA1*01 allele is over-represented alongside HLA-DPB1*0202 in
Japanese KFD patients. As with HLA-DPB1, this is a germline susceptibility
association, not a causal Mendelian mutation.
gene_term:
preferred_term: HLA-DPA1
term:
id: hgnc:4938
label: HLA-DPA1
variant_origin: GERMLINE
relationship_type: RISK_FACTOR
evidence:
- reference: PMID:10519361
reference_title: "DNA typing of HLA class II genes (HLA-DR, -DQ and -DP) in Japanese patients with histiocytic necrotizing lymphadenitis (Kikuchi's disease)."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
HLA class II genes of HNL and the incidence of HNL in Asian countries,
including Japan, might have a positive relationship to DPA1*01 and
DPB1*0202 allele
explanation: >-
Links the HLA-DPA1*01 (and DPB1*0202) alleles to HNL and to its higher
incidence in Asian populations, supporting HLA-DPA1 as a susceptibility
locus.
biochemical:
- name: Leukopenia
notes: >-
A reduced peripheral white blood cell count is the commonest laboratory
abnormality; atypical lymphocytes may be present.
evidence:
- reference: PMID:37383134
reference_title: "Kikuchi-Fujimoto disease: A comprehensive review."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
weight loss, splenomegaly, leucopenia, and elevated erythrocyte
sedimentation rate feature in severely affected patients
explanation: Documents leukopenia as a laboratory abnormality in KFD.
- name: Antinuclear antibody status
notes: >-
ANA is usually negative at baseline in isolated KFD; ANA positivity (and
low C4) is associated with recurrence and with an intermediate status
toward overt autoimmune disease such as SLE, so it is an important
prognostic and surveillance marker.
evidence:
- reference: PMID:39961627
reference_title: "Kikuchi-Fujimoto disease: investigating comprehensive clinicopathological features and risk factors for recurrence."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Recurrence was associated with lower C4 levels (P = 0.038) and higher
antinuclear antibody (ANA) rates (P = 0.007) compared to transient
disease.
explanation: >-
Establishes ANA positivity and low C4 as markers associated with KFD
recurrence and evolving autoimmunity.
histopathology:
- name: Paracortical Necrosis with Karyorrhexis
finding_term:
preferred_term: Paracortical necrosis with karyorrhectic (nuclear) debris
term:
id: NCIT:C36184
label: Necrosis
diagnostic: true
description: >-
The diagnostic core of KFD is paracortical (T-zone) foci of necrosis with
abundant apoptotic karyorrhectic (nuclear) debris, an infiltrate of
crescentic histiocytes (CD68-positive, myeloperoxidase-positive),
plasmacytoid dendritic cells, and CD8-positive T cells, with a
characteristic absence of neutrophils and a paucity of plasma cells and
B cells. Fine-needle aspiration is often inadequate; excisional lymph-node
biopsy is required.
evidence:
- reference: PMID:28613580
reference_title: "Kikuchi-Fujimoto Disease."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
An excisional lymph node biopsy is imperative for confirming a definitive
diagnosis, revealing a deficiency of neutrophils and eosinophils.
explanation: >-
Documents the diagnostic requirement for excisional biopsy and the
characteristic deficiency of neutrophils, a key distinguishing histologic
feature of KFD.
- reference: PMID:28613580
reference_title: "Kikuchi-Fujimoto Disease."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Immunohistochemistry will demonstrate histiocytes positive for
myeloperoxidase and CD68, T cells positive for CD8, and a minimal
presence of B cells.
explanation: >-
Establishes the characteristic immunophenotype: CD68/MPO-positive
histiocytes, CD8-positive T cells, and scant B cells.
diagnosis:
- name: Excisional lymph node biopsy
description: >-
Definitive diagnosis is made by excisional lymph-node biopsy showing the
characteristic histopathology; fine-needle aspiration is often
nondiagnostic. Biopsy is essential to exclude lymphoma and to distinguish
KFD from lupus lymphadenitis.
evidence:
- reference: PMID:28613580
reference_title: "Kikuchi-Fujimoto Disease."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
An excisional lymph node biopsy is imperative for confirming a definitive
diagnosis, revealing a deficiency of neutrophils and eosinophils.
explanation: Documents excisional lymph-node biopsy as the definitive diagnostic test.
- name: Exclusion of lymphoma and SLE
description: >-
KFD must be distinguished from non-Hodgkin lymphoma and from systemic lupus
erythematosus lymphadenitis; the presence of hematoxylin bodies favors SLE
lymphadenitis over KFD.
evidence:
- reference: PMID:28613580
reference_title: "Kikuchi-Fujimoto Disease."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Distinguishing Kikuchi-Fujimoto disease from lymphomas and infectious
etiologies is critical
explanation: >-
Establishes the diagnostic imperative of excluding lymphoma and
infectious mimics.
differential_diagnoses:
- name: Systemic lupus erythematosus (lupus lymphadenitis)
description: >-
Lupus lymphadenitis can be histologically similar to KFD, but the presence
of hematoxylin bodies (and abundant plasma cells) favors SLE. SLE is the
autoimmune disease most strongly associated with KFD, and KFD patients
require follow-up because they may develop SLE.
distinguishing_features:
- "KFD: paucity of plasma cells, no hematoxylin bodies, absence of neutrophils"
- "SLE lymphadenitis: hematoxylin bodies present, abundant plasma cells"
evidence:
- reference: PMID:28613580
reference_title: "Kikuchi-Fujimoto Disease."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
the presence of hematoxylin bodies in SLE lymphadenitis aids in its
distinction from Kikuchi-Fujimoto disease
explanation: >-
Identifies hematoxylin bodies as the key histologic feature
distinguishing SLE lymphadenitis from KFD.
disease_term:
preferred_term: systemic lupus erythematosus
term:
id: MONDO:0007915
label: systemic lupus erythematosus
- name: Non-Hodgkin lymphoma
description: >-
Nodal lymphoma is the most important mimic of KFD because misdiagnosis can
lead to unnecessary chemotherapy. KFD lacks a clonal lymphoid population;
absence of clonal rearrangement and the characteristic histiocytic
necrotizing pattern distinguish KFD from lymphoma.
distinguishing_features:
- "KFD: reactive polyclonal infiltrate, CD8 T cells, histiocytic karyorrhectic necrosis, no clonality"
- "Non-Hodgkin lymphoma: clonal lymphoid population, effacement of architecture"
evidence:
- reference: PMID:37383134
reference_title: "Kikuchi-Fujimoto disease: A comprehensive review."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
It is often mistaken for non-Hodgkin lymphoma
explanation: >-
Documents non-Hodgkin lymphoma as the principal misdiagnosis of KFD,
making it the key differential.
disease_term:
preferred_term: non-Hodgkin lymphoma
term:
id: MONDO:0018908
label: non-Hodgkin lymphoma
- name: Tuberculous lymphadenitis
description: >-
Tuberculous (and other granulomatous infectious) lymphadenitis enters the
differential for necrotizing lymphadenopathy; KFD characteristically lacks
well-formed granulomas and neutrophils and shows apoptotic karyorrhexis
instead of caseating necrosis with acid-fast bacilli.
distinguishing_features:
- "KFD: apoptotic karyorrhexis, no granulomas, no neutrophils, culture/AFB negative"
- "Tuberculous lymphadenitis: caseating granulomas, acid-fast bacilli"
evidence:
- reference: PMID:37383134
reference_title: "Kikuchi-Fujimoto disease: A comprehensive review."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
lupus lymphadenitis, cat-scratch disease, Sweet's syndrome, Still's
disease, drug eruptions, infectious mononucleosis, and viral or
tubercular lymphadenitis are other common differentials
explanation: >-
Lists tubercular (and other infectious) lymphadenitis among the common
differentials of KFD.
disease_term:
preferred_term: lymph node tuberculosis
term:
id: MONDO:0005831
label: lymph node tuberculosis
- name: Cat-scratch disease
description: >-
Cat-scratch disease (Bartonella henselae) causes granulomatous, often
suppurative regional lymphadenitis with neutrophilic microabscesses, in
contrast to the neutrophil-poor apoptotic necrosis of KFD.
distinguishing_features:
- "KFD: apoptotic karyorrhexis, absence of neutrophils"
- "Cat-scratch disease: stellate necrotizing granulomas with neutrophilic microabscesses, Bartonella exposure"
evidence:
- reference: PMID:37383134
reference_title: "Kikuchi-Fujimoto disease: A comprehensive review."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
lupus lymphadenitis, cat-scratch disease, Sweet's syndrome, Still's
disease, drug eruptions, infectious mononucleosis, and viral or
tubercular lymphadenitis are other common differentials
explanation: Lists cat-scratch disease among the common differentials of KFD.
disease_term:
preferred_term: cat-scratch disease
term:
id: MONDO:0005692
label: cat-scratch disease
- name: Rosai-Dorfman disease
description: >-
Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) is
the other histiocytic lymphadenopathy in the differential. It is
distinguished by S100-positive histiocytes exhibiting emperipolesis (the
non-destructive engulfment of intact lymphocytes), which is absent in KFD;
KFD instead shows CD8 T-cell-driven apoptotic karyorrhectic necrosis
without emperipolesis.
distinguishing_features:
- "KFD: apoptotic karyorrhectic necrosis, CD8 T cells, no emperipolesis, S100 variable"
- "Rosai-Dorfman disease: emperipolesis, S100-positive CD1a-negative histiocytes, massive painless lymphadenopathy"
disease_term:
preferred_term: Rosai-Dorfman disease
term:
id: MONDO:0006412
label: sinus histiocytosis with massive lymphadenopathy
treatments:
- name: Supportive care
description: >-
Because KFD is self-limiting, most patients require only supportive care
(hydration, antipyretics, monitoring) and reassurance while the disease
resolves spontaneously.
treatment_term:
preferred_term: Supportive Care
term:
id: NCIT:C15747
label: Supportive Care
evidence:
- reference: PMID:28613580
reference_title: "Kikuchi-Fujimoto Disease."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
The management of Kikuchi-Fujimoto disease primarily involves supportive
care for patients, with the use of corticosteroids and immunosuppression
reserved for cases of severe or recurrent disease.
explanation: >-
Establishes supportive care as the primary management, with
corticosteroids/immunosuppression reserved for severe or recurrent
disease.
- name: NSAIDs and analgesics
description: >-
Non-steroidal anti-inflammatory drugs (such as ibuprofen), antipyretics,
and analgesics are used for symptom control (fever and painful nodes) in
mild disease.
therapeutic_modality: SMALL_MOLECULE
treatment_term:
preferred_term: Pharmacotherapy
term:
id: NCIT:C15986
label: Pharmacotherapy
therapeutic_agent:
- preferred_term: ibuprofen (representative NSAID)
term:
id: CHEBI:5855
label: ibuprofen
evidence:
- reference: PMID:36451640
reference_title: "Kikuchi-Fujimoto Disease: A Rare Cause of Pyrexia of Unknown Origin and Cervical Lymphadenopathy."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Supportive treatment includes antipyretics, non-steroidal
anti-inflammatory drugs, and corticosteroids.
explanation: >-
Documents NSAIDs and antipyretics as supportive treatment for KFD.
- name: Systemic corticosteroids
description: >-
Systemic corticosteroids (e.g., prednisolone) hasten resolution and are
reserved for severe, extranodal, or recurrent disease.
therapeutic_modality: SMALL_MOLECULE
treatment_term:
preferred_term: Pharmacotherapy
term:
id: NCIT:C15986
label: Pharmacotherapy
therapeutic_agent:
- preferred_term: prednisolone (representative corticosteroid)
term:
id: CHEBI:8378
label: prednisolone
evidence:
- reference: PMID:28613580
reference_title: "Kikuchi-Fujimoto Disease."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
the use of corticosteroids and immunosuppression reserved for cases of
severe or recurrent disease
explanation: >-
Documents systemic corticosteroids reserved for severe or recurrent KFD.
- name: Hydroxychloroquine
description: >-
Hydroxychloroquine is used as an immunomodulatory agent in recurrent or
corticosteroid-dependent disease and in cases with autoimmune (lupus)
overlap.
therapeutic_modality: SMALL_MOLECULE
treatment_term:
preferred_term: Pharmacotherapy
term:
id: NCIT:C15986
label: Pharmacotherapy
therapeutic_agent:
- preferred_term: hydroxychloroquine
term:
id: CHEBI:5801
label: hydroxychloroquine
evidence:
- reference: PMID:37383134
reference_title: "Kikuchi-Fujimoto disease: A comprehensive review."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Its treatment with systemic corticosteroids, hydroxychloroquine, or
antimicrobial agents mostly remains empirical.
explanation: >-
Documents hydroxychloroquine (with corticosteroids) as an empirical
treatment for KFD.
discussions:
- discussion_id: kfd_sle_association
kind: INTERPRETATION
status: OPEN
prompt: >-
How should the strong but longitudinal association between Kikuchi-Fujimoto
disease and systemic lupus erythematosus be modeled and surveilled?
rationale: >-
KFD has a well-recognized, complex association with systemic lupus
erythematosus (SLE): SLE may precede, develop after, or occur concurrently
with KFD. Recurrent KFD, higher ANA positivity, and low C4 mark an
intermediate status toward overt autoimmune disease, so KFD patients
warrant long-term follow-up with ANA and complement monitoring for evolving
SLE. This is modeled as a discussion rather than a structured comorbidity
because KFD-SLE is a longitudinal association/overlap rather than a fixed
co-occurrence.
evidence:
- reference: PMID:37383134
reference_title: "Kikuchi-Fujimoto disease: A comprehensive review."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Both Kikuchi-Fujimoto disease and systemic lupus erythematosus share an
obscure and complex relationship as systemic lupus erythematosus may
occasionally precede, develop subsequently, or sometimes be associated
concurrently with Kikuchi-Fujimoto disease.
explanation: >-
Documents the temporal complexity of the KFD-SLE association, motivating
SLE surveillance.
- reference: PMID:39961627
reference_title: "Kikuchi-Fujimoto disease: investigating comprehensive clinicopathological features and risk factors for recurrence."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
patients with recurrent KFD represent an intermediate status between
those with transient KFD and those with overt autoimmune disease
explanation: >-
Supports recurrent KFD as an intermediate state toward autoimmune
disease, reinforcing the need for autoimmune surveillance.
- discussion_id: kfd_no_animal_model
kind: KNOWLEDGE_GAP
status: OPEN
prompt: >-
Is there a validated animal or in vivo model that recapitulates
Kikuchi-Fujimoto disease?
rationale: >-
There is no established animal (mouse, rat, zebrafish, or other in vivo)
model that recapitulates KFD. Mechanistic understanding rests almost
entirely on immunohistochemistry and in-situ studies of human lymph-node
tissue, so causal mechanisms remain hypotheses. All KFD mechanistic
evidence in this entry is therefore tagged HUMAN_CLINICAL or IN_VITRO
(tissue-based); none is MODEL_ORGANISM because no faithful animal model
data exist.
attaches_to:
- CD8 T-Cell- and Histiocyte-Mediated Apoptotic Necrosis
classifications:
harrisons_chapter:
- classification_value: IMMUNE_RHEUMATOLOGIC
evidence:
- reference: PMID:39961627
reference_title: "Kikuchi-Fujimoto disease: investigating comprehensive clinicopathological features and risk factors for recurrence."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
patients with recurrent KFD represent an intermediate status between
those with transient KFD and those with overt autoimmune disease
explanation: >-
The immune-mediated nature and autoimmune (SLE) overlap support an
immune/rheumatologic placement.
- classification_value: INFECTIOUS_DISEASES
evidence:
- reference: PMID:36451640
reference_title: "Kikuchi-Fujimoto Disease: A Rare Cause of Pyrexia of Unknown Origin and Cervical Lymphadenopathy."
supports: PARTIAL
evidence_source: HUMAN_CLINICAL
snippet: >-
Although the clinical and histopathological features point to a viral
etiology, this hypothesis has not been proven yet.
explanation: >-
A hypothesized (unproven) infectious/viral trigger supports a secondary
infectious-disease placement, framed as hypothesis rather than
established cause.
notes: >-
Curation informed by a claude_code deep-research report
(research/Kikuchi-Fujimoto_Disease-deep-research-claude_code.md) used as
leads only. All evidence snippets were independently verified as exact
substrings of cached PubMed abstracts. NEC preflight: MONDO:0018864 confirmed
via OAK as Kikuchi-Fujimoto disease / histiocytic necrotizing lymphadenitis
(is_a lymphadenitis), and the entry is anchored on that identity, not on
Rosai-Dorfman disease, lymphoma, or SLE lymphadenitis. Two deep-research
quotes attributed to PMID:37383134 (a Fas-FasL sentence and an
IFN-alpha/2',5'-oligoadenylate sentence) were NOT present in that paper's
abstract, so those claims were re-sourced to PMID:9839173 (Fas/perforin
apoptosis) and PMID:19141377 (MxA/interferon dendritic-cell signature)
whose abstracts do contain verbatim support. No GeneReviews chapter exists
for KFD (PubMed "Kikuchi-Fujimoto GeneReviews[All Fields]" returns zero
results), as expected for a non-Mendelian disorder; the HLA-DPB1*0202 /
HLA-DPA1*01 signal is modeled as a germline susceptibility association
(relationship_type RISK_FACTOR), not a causal Mendelian gene, and no
unproven viral cause is asserted as established fact. No mechanism module was
a genuine fit, so none is declared. No validated animal model exists (tracked
as a KNOWLEDGE_GAP discussion).
Overview. Kikuchi-Fujimoto disease (KFD), synonymously histiocytic necrotizing lymphadenitis (HNL), is a rare, benign, usually self-limiting cause of subacute regional (typically cervical) lymphadenopathy with fever. It was described independently in Japan in 1972 by Masahiro Kikuchi and by Yoshiro Fujimoto. Diagnosis is exclusively histopathological — the clinical and imaging picture is nonspecific and closely mimics both lymphoma and lupus lymphadenitis, so an excisional lymph-node biopsy is essential (PMC11592699, MDPI Diseases 2024; comprehensive review, PMID 37383134 / PMC10294163).
"A rare form of necrotizing lymphadenitis, is an uncommon, benign, self-limiting disorder of obscure etiology." — comprehensive review (PMID 37383134)
Key identifiers.
| System | ID |
|---|---|
| MONDO | MONDO:0018864 |
| Orphanet | ORPHA:50918 |
| ICD-11 | 4B2Y (other/unspecified) — often coded under nonspecific lymphadenitis |
| ICD-10 | I88.1 (chronic lymphadenitis) |
| MeSH | D020042 ("Histiocytic Necrotizing Lymphadenitis") |
| UMLS | C0398367 |
| GARD | 6834 |
| MedDRA | 10069070 |
(Identifiers compiled from Orphanet/NORD/EBI-OLS listings — please re-verify MONDO/ICD mappings against the local sqlite:obo:mondo adapter before committing, per the NEC preflight in CLAUDE.md.)
Synonyms / alternative names: Kikuchi disease; Kikuchi-Fujimoto syndrome; histiocytic necrotizing lymphadenitis (HNL); necrotizing lymphadenitis; subacute necrotizing lymphadenitis.
Data provenance: Disease-level, aggregated from case series and reviews (not EHR/individual-patient registries). No dedicated large prospective registry exists.
The cause is unknown. Two non-exclusive hypotheses dominate: (a) an infectious trigger (most often viral) and (b) an exaggerated, self-limited autoimmune/T-cell–mediated response in a genetically susceptible host — essentially a transient "SLE-like" reaction.
Infectious/viral hypothesis. Many agents have been associated (temporally, serologically, or by case report) but none proven causal: EBV, HSV, VZV, HHV-6/7/8, parvovirus B19, paramyxovirus, parainfluenza, rubella, CMV, and — more recently — SARS-CoV-2 infection and post-COVID-vaccination cases (PMC11592699). Support for a viral component comes from raised IFN-α and its downstream marker 2′,5′-oligoadenylate synthetase, plus tubuloreticular structures in the cytoplasm of stimulated lymphocytes/histiocytes/endothelium — an interferon-signature also seen in SLE (PMID 37383134).
"Increased levels of IFN-α and its stimulators including 2′,5′-oligoadenylate synthetase, and tubuloreticular structures in the cytoplasm of stimulated lymphocytes." — PMID 37383134
Autoimmune hypothesis. KFD is postulated to be "a self-limiting SLE-like autoimmune disorder caused by virus-infected transformed lymphocytes" triggering a cell-mediated response "to a variety of antigens in genetically susceptible people" (PMC11592699).
Genetic risk factors. No causal Mendelian gene. The principal susceptibility signal is HLA class II, notably HLA-DPA1 and HLA-DPB1 alleles that are far more common in East Asians than in Europeans. A Japanese case-control study (86 KFD patients vs 525 controls) found DPA1*01 and DPB1*0202 significantly over-represented in KFD; these alleles are common in Korea (~9.9%) and Japan (~4.5%) but rare in French (~0.4%) and Italian (~0.8%) populations — plausibly explaining the Asian predominance (summarized in PMC11592699). A 2023 report described familial KFD in HLA-partially-matched siblings both carrying DPB1*0202 (PMC10803893). RNA- and exome-sequencing efforts have begun to nominate additional candidate genes (ScienceDirect S0890850821000359).
Environmental / lifestyle risk factors: young age and Asian ancestry are the strongest demographic risks; female sex from the 30s onward. No confirmed occupational, toxic, dietary, or smoking association.
Protective factors: none established (genetic or environmental).
Gene–environment interaction (working model): an environmental antigen (virus) in a host carrying permissive HLA-DP alleles drives an over-exuberant CD8⁺ T-cell response that produces the necrotizing lesion, then self-terminates.
For a KB entry, the core phenotype bundle (with suggested HPO terms and approximate frequencies from case series) is:
| Phenotype | Type | Frequency | Suggested HPO |
|---|---|---|---|
| Cervical lymphadenopathy (usually unilateral, posterior, tender) | Sign | ~virtually all cases; cervical ~85% | HP:0002726 (Abnormal cervical lymph node morphology) / HP:0002716 (Lymphadenopathy) |
| Fever (intermittent, low-grade) | Symptom | ~30–55% | HP:0001945 (Fever) |
| Leukopenia | Lab | ~18–50% (commonest lab abnormality) | HP:0001882 (Leukopenia) |
| Elevated ESR | Lab | ~16–70% | HP:0003565 (Elevated erythrocyte sedimentation rate) |
| Elevated LDH | Lab | frequent | HP:0025435 (Increased circulating lactate dehydrogenase) |
| Anemia (chronic disease) | Lab | ~9–23% | HP:0001903 (Anemia) |
| Cutaneous lesions (facial erythema, papules/plaques, morbilliform/acneiform) | Sign | ~30–40% | HP:0000988 (Skin rash) |
| Night sweats | Symptom | common | HP:0000989? (use HP:0030166 Night sweats) |
| Weight loss | Symptom | occasional | HP:0001824 (Weight loss) |
| Splenomegaly | Sign | occasional (severe cases) | HP:0001744 (Splenomegaly) |
| Hepatomegaly / elevated transaminases | Sign/Lab | occasional | HP:0002240 / HP:0002910 |
| Arthralgia/arthritis | Symptom | occasional | HP:0002829 (Arthralgia) |
| Aseptic meningitis / CNS involvement | Sign | rare (extranodal) | HP:0033146 (Aseptic meningitis) / HP:0002383 (Encephalitis) |
| Atypical lymphocytosis | Lab | ~25–30% | HP:0031392 (Atypical lymphocytes) |
Characteristics. Onset is subacute over 1–4 weeks; symptom duration averages ~2–3 weeks but can persist — one Japanese cohort reported a mean symptomatic duration of 71.5 days (PMID 39961627 / PMC12129606). Severity is usually mild–moderate and self-limited; a minority have severe systemic/extranodal disease. Course is self-limited but occasionally recurrent (see §8, §11).
"Fever (55.2%) was the most common clinical manifestation. Leukopenia (49.3%) was the commonest reported laboratory abnormality." — cervical-KFD retrospective (n=134), PMC10446312
Quality-of-life impact: generally limited given self-resolution, but the diagnostic odyssey (fear of lymphoma), painful nodes, prolonged fever, and recurrence-related anxiety are the main burdens. No KFD-specific QoL instrument exists.
Suggested gene descriptors (HGNC): hgnc:4938 (HLA-DPA1), hgnc:4940 (HLA-DPB1). Because casing in this repo is lowercase hgnc:.
Causal chain (best-supported hypothesis):
Cell types involved (suggested CL terms): - CD8⁺ cytotoxic T cell — CL:0000794 - Plasmacytoid dendritic cell — CL:0000784 (CD123⁺/CD303⁺; a defining infiltrate) - Myeloid/immature dendritic cell — CL:0000840 (immature) / CL:0000451 - Histiocyte / macrophage — CL:0000235 (CD68⁺, CD163⁺, MPO⁺, lysozyme⁺) - Immunoblast (large transformed lymphoid cell) — CL:0000653? (use B/T immunoblast as available)
Immunophenotype of the diagnostic lesional cells (Pileri/Facchetti-type analysis, PMID 19141377): the "plasmacytoid" mononuclear cells are two populations of immature dendritic cells — myeloid DCs (CD68⁺, CD1c/BDCA-1⁺, CD13/CD33⁺) and plasmacytoid DCs (CD303/BDCA-2⁺, CD123⁺), strongly MxA⁺ (IFN-α–inducible) and fascin-negative (immature).
"The morphologically distinctive mononuclear cells in lesional areas consisted of 2 populations of immature DCs: myeloid DCs …" — PMID 19141377
Suggested GO biological-process terms: - GO:0006915 apoptotic process (INCREASED) - GO:0097191 extrinsic apoptotic signaling pathway / GO:0036337 Fas signaling (INCREASED) - GO:0060337 type I interferon signaling pathway (INCREASED) - GO:0001913 T cell–mediated cytotoxicity (INCREASED) - GO:0006909 phagocytosis (INCREASED) - GO:0002456 T cell–mediated immunity (INCREASED)
Immune involvement: this is fundamentally an immune-mediated disease (cytotoxic T cell + interferon + dendritic-cell axis), sitting on a spectrum toward SLE — hence the overlapping interferon signature and the clinical progression of a subset to lupus. Molecular profiling beyond IHC (bulk transcriptomics/single-cell/proteomics) is sparse; no metabolomic or lipidomic signature is defined.
Definitive test: excisional lymph-node biopsy with histopathology (FNA is often nondiagnostic and risks missing lymphoma).
"With a reported fatality rate of 2.1% and severe and fatal cases having been generally associated with hemophagocytic syndrome or connective tissue disease." — PMID 37383134
No approved disease-specific therapy; management is empirical and largely supportive — the disease self-resolves (PMC11592699; PMID 37383134).
| Line | Intervention | Notes | Suggested MAXO/NCIT |
|---|---|---|---|
| Mild disease | NSAIDs / analgesics / antipyretics | Symptom control (fever, node pain) | MAXO:0000058 (NSAID therapy) / NCIT:C15986 Pharmacotherapy + CHEBI agent |
| Moderate–severe / extranodal / relapsing | Systemic corticosteroids (e.g., prednisolone) | Rapid symptom resolution; reserved for severe/extranodal disease | NCIT:C15986 Pharmacotherapy + therapeutic_agent NCIT:C2322 Corticosteroid (or CHEBI prednisolone) |
| Recurrent / steroid-dependent / autoimmune-leaning | Hydroxychloroquine | Immunomodulatory; used in recurrent/lupus-associated cases | NCIT:C15986 + CHEBI:5801 (hydroxychloroquine) |
| Severe/refractory or HLH | IVIG, other immunomodulators; HLH-directed therapy | Case-based | MAXO:0000841 (immunoglobulin therapy) / supportive care MAXO:0000950 |
| Supportive | Supportive care | Hydration, monitoring | MAXO:0000950 supportive care |
KNOWLEDGE_GAP discussion node in the KB entry.⚠️ Follow the dismech SOP: run
just fetch-reference PMID:37383134(etc.), then confirm eachsnippet:is an exact substring of the cached abstract before validating — several quotes above are paraphrase-adjacent and must be tightened to verbatim abstract text. Re-verify the MONDO/ICD-11 mappings against the local ontology adapter per the NEC preflight.
Sources: - Kikuchi-Fujimoto disease: A comprehensive review — PMID 37383134 / PMC10294163 - Kikuchi–Fujimoto Disease: A Case Series and Review — PMC11592699 (MDPI Diseases 2024) - Clinicopathological features and risk factors for recurrence — PMID 39961627 / PMC12129606 (Histopathology 2025) - Retrospective study of 134 cervical KFD patients — PMC10446312 - Lesional cells exhibit an immature dendritic cell phenotype — PMID 19141377 - KFD in HLA partially-matched siblings (familial susceptibility) — PMC10803893 - Genes associated with KFD via RNA/exome sequencing — ScienceDirect 2021 - SARS-CoV-2 triggering KFD — PMC10077921 - KFD + SLE + HLH case report — PMC6551871 - Kikuchi-Fujimoto Disease: A Review — Arch Pathol Lab Med 2018 - Orphanet ORPHA:50918 · NORD MONDO listing · GARD 6834 - StatPearls: Kikuchi-Fujimoto Disease — NBK430830