Kikuchi necrotizing adenitis

eMedicine:3663

MeSH:D020042

Wikipedia

Digital cases

 HPC:339 : Kikuchi adenitis (necrotizing histiocytic lymphadenitis)
 JRC:991 : Kikuchi’s lymphadenitis in a 27 y/o female.
 JRC:15279 : Kikuchi’s lymphadenitis in a 27 y/o female.

Definition: Kikuchi’s disease, or necrotizing histiocytic lymphadenitis, is a rare disease that presents predominantly in young women in their 20s and 30s from the Far East.

Kikuchi-Fujimoto disease (KFD), first described independently by Kikuchi and Fujimoto in 1972, is a subacute necrotizing lymphadenitis of unknown cause.

Although most frequent in young Asian women, KFD has a worldwide distribution.

Clinically, KFD is characterized by lymphadenitis of one or more lymph nodes, predominantly in the posterior cervical region, fever, and leukopenia in up to 50% of cases.

Extranodal manifestations can occur, especially skin lesions and aseptic meningitides. Diagnosis is usually confirmed by analysis of samples from an excisional biopsy of the affected nodes.

The course is usually benign with resolution in a few months with the use of antiinflammatory drugs. Regular follow-up is required because SLE may develop several years after the onset of Kikuchi-Fujimoto disease.

Synopsis

 cervical lymphadenopathy
 cortical and paracortical areas of the node
 focal necrosis predominantly in the paracortical region
 abundant karyorrhectic debris
 atypical mononuclear cells around the necrotic zone

• crescent-shaped histiocytes
• plasmacytoid monocytes
• small lymphocytes, mostly CD3(+)/CD8(+)
• immunoblasts, mostly CD3(+)/CD8(+)
• +/- intact lymph node capsule
• absence of neutrophils
• paucity of plasma cells

NB: KFD has been classified into three histological subtypes and is thought to progress from the proliferative type (> 50%) to the necrotizing type (30%) and finally resolve into the xanthomatous type ( < 20%).

Associations

 enthesitis-related arthritis (18470474)
 systemic lupus erythematossus (18465190, 18304938)
 systemic juvenile idiopathic arthritis (14739479)
 autoimmune hepatitis (18090996)
 extreme leukopenia and thrombocytopenia (18090944)
 macrophagic activation (hemophagocytic lymphohistiocytosis) (18067022, 17939267)
 parvovirus B19 (17931112, 1845362, 1656832)

Differential diagnosis

 malignant lymphoma
 infectious diseases

• toxoplasmatic lymphadenitis
• tuberculous lymphadenitis
• cat scratch disease

 systemic lupus erythematosus (SLE)

• lupic lymphadenitis

Associations

 t(2:16) chromosomal translocation (17405981)

See also

 necrotizing adenitis