(Histiocytic necrotizing lymphadenitis)

Histiocytic necrotizing lymphadenitis. Female predominance in Chinese adults, but male predominance in African children. Sometimes associated to homozygous drepanocytosis.  Benign disorder characterized by generalized lymph nodes (but predominantly in the posterior cervical region) (90 %)  with fever (50 %). Lymph nodes are hard, mobile and painful.

Sometimes associated with:

-        important weight loss (50 %)

-        cytopenia

-        hepatosplenomegaly

-        morbilliform urticaria -like rash

-        nocturnal sweats.

Evolution on 3 to 24 months. Female adults in Asia, but male predominance in African children. Sometimes associated with homozygous sickle cell disease.

The clinical picture and the evolution advocate for a hyperimmune process of infectious origin. There is a strong association with the further development of a systemic erythematosus lupus in adulthood and with a familial hemophagocytic lymphohistiocytosis in childhood.

The lymph node biopsy gives the diagnostic: preserved lymph node architecture and coagulated necrosis in cortical and paracortical regions. Three histological forms have been described: necrotizing, proliferative (or pseudo-tumor) and xanthomatous.

Differential diagnosis in children and adolescents:

-        infectious lymphadenopathy: EBV, tuberculosis, toxoplasmosis, cat-scratch disease (CSD)

-        tumoral lymph nodes: lymphoma (keep in mind that PET scan is also positive)

-        inflammatory adenitis: Still's disease, Kawasaki disease

-        autoimmune disease: systemic lupus erythematosus

-        other: Castelman's disease, Rosai-Dorfman syndrome.

Treatment: surgical resection, either NSAIDs or corticosteroids. Similar to lupus treatment is sometimes necessary in  highly systemic forms.

Anesthetic implications:

not specific, but risk of obstruction of the airway or trismus in case of significant lymphadenopathy. Check chest XRay. Sometimes anesthesia in the context of the diagnosis of a lymphoma.

References : 

-        Marec C, Picherot G, Kandel C, Caquard M. Une cause rare chez l’enfant de poly-adénopathie fébrile : la maladie de Kikuchi-Fujimoto. Arch Pédiatr 2012 ; 19 : 38-41.

-        Goffin L, Thomée C, Dehou M-F, Ferster A. 
Maladie de Kikuchi : à propos de 3 cas pédiatriques. 
Percentile 2012

-         Rossetti E, Appierto L, Bianchi R, Picardo S. 
Kikuchi-Fujimoto disease and life-threatening upper airway obstruction. 
Pediatr Anesth 2015; 25: 864-5.

-        Escudier A, Courbage S, Meignin V, Abbou S et al.
La maladie de Kikuchi-Fujimoto : un diagnostic différentiel méconnu du lymphome de l’adolescent.
Arch Pédiatr 2017 ; 24 : 471-5.

-        Iio K, Matsuoka K.
Visible lymphadenopathy in Kikuchi-Fujimoto disease.
J Pediatr 2020; 226:300

Updated: October 2020