Skip to content

Advertisement

  • Poster presentation
  • Open Access

Various aspects of Kikuchi disease in three children: systemic or self-limited disease?

  • 1Email author,
  • 1,
  • 1,
  • 1,
  • 2,
  • 3,
  • 3 and
  • 1
Pediatric Rheumatology20119 (Suppl 1) :P235

https://doi.org/10.1186/1546-0096-9-S1-P235

  • Published:

Keywords

  • Lymph Node
  • Young Adult
  • Pediatric Patient
  • Histological Examination
  • Favorable Outcome

Kikuchi’s disease (KD), or histiocytic necrotizing lymphadenitis, is a rare benign and self-limited disease involving young adults, predominantly females. It is rarely described in children. It is characterized by localized lymphadenopathy, often associated with fever and systemic symptoms. The diagnosis is based on histological examination of lymph node biopsy. The disease usually resolves spontaneously over a period of several weeks to months. In some cases, KD reveals or evolves into a systemic lupus, reason why long term follow-up is recommended.

Three cases of pediatric KD are presented in Table 1.

Table 1

 

Patient 1

Patient 2

Patient 3

Age (years)

5

17

9

Underlying disease

Non complicated sickle cell anemia

Severe sickle cell anemia

None

Symptoms

- Left cervical lymphadenopathy

- Intermittent fever

- Intermittent fever

- Maculo-papules

- Fatigue

- Painful cervical and inguinal adenopathies

- Headaches

- Polyarthritis

- Prolonged fever

- Intermittent tonsillitis

- Bilateral painful cervical lymph nodes

Biological features

- Mild inflammation

- Elevated LDH

- Marked inflammation

- Pancytopenia

- Auto-immune anemia

- Auto-immune hepatitis

- No ANA

- Marked inflammation

- Leucopenia

Biopsy

Cervical lymph node excisional biopsy

Cervical lymph node excisional biopsy

Tonsillectomy

Treatment

None

- Steroids for 7 months

- Azathioprine

- Methotrexate

None

Outcome

Spontaneous resolution after 4 months

Progressive regression of clinical and biological signs with resolution of auto-immunity

Spontaneous resolution 2 months after tonsillectomy

In conclusion, KD is rarely observed in children, has various presentations but usually favorable outcome. This small cohort of pediatric patients illustrates this diversity: one of them presented with marked systemic symptoms, suggesting SLE but resolving after prolonged corticotherapy, while the others had a more benign course with spontaneous resolution after excision. Association with sickle cell disease has not been described and, to our best knowledge, this is the first case diagnosed on tonsil examination.

Authors’ Affiliations

(1)
Department of PediatryGoffin et al; licensee BioMed Central Ltd, Hôpital Universitaire Des Enfants Reine Fabiola, Brussels, Belgium
(2)
Department of Pediatry, Centre Hospitalier de Luxembourg, Luxemburg
(3)
Department of pathology, CHU Brugmann, Brussels, Belgium

Copyright

Advertisement