Kikuchi's disease in Asian children

Objective. Kikuchi's disease (KD), or histiocytic necrotizing lymphadenitis, is a unique form of self-limiting lymphadenitis and typically affects the head and neck regions. It usually occurs in young adults and has a female predilection. The aim of this study was to review the authors' institutional experience with KD in children over a 16-year period. Methods. Between January 1986 and May 2002, a total of 23 patients who were younger than 16 years underwent cervical lymph node biopsies and received a diagnosis of KD. Clinical features, laboratory values, pathologic parameters, specific characteristics of our pediatric patients, and long-term follow-up results are discussed. The follow-up period averaged 8 years. Results. There were 8 girls and 15 boys with a mean age of 12.8. All 23 patients had affected cervical lymph nodes located in the posterior cervical triangle, and 2 cases additionally had affected nodes in the anterior triangle. Cervical lymph nodes were affected unilaterally in 82.6% (19) and bilaterally in 17.4% (4) of these patients. The dimensions of the affected lymph nodes were commonly in the range of 0.5 to 3 cm (52.2%; 12 of 23) and 3 to 6 cm (39.1%; 9 of 23). In 2 (8.7%) patients, the size of the enlarged lymph nodes reached >6 cm. Leukopenia was observed in 5 (21.7%) patients, and fever was observed in 7 (30.4%) patients. One child with KD developed systemic lupus erythematosus 5 years later. The cervical lymphadenopathy usually resolved itself without any medical treatment within 6 months after definite diagnosis was made. Neither recurrence nor persisting KD has since been noted. Conclusion. KD, although rare, should be part of the differential diagnosis for posterior cervical lymphadenopathy in children, especially in patients of Asian descent. In our pediatric series, KD demonstrated a male predominance, which is in contrast to previously reported adult series demonstrating a female predominance. The children with KD require a systemic survey and regular follow-up for several years to rule out the development of systemic lupus erythematosus.