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Characteristics of Kikuchi-Fujimoto disease in children compared with adults
Tae Yeun Kim1, Kee-Soo Ha, Yunkyung Kim
1Korea University School of Medicine, 73 Inchon-ro, Sungbuk-gu, Seoul, 136-705, Republic of Korea.
Insights
Kikuchi-Fujimoto disease (KFD) in children presents differently than in adults. While cervical lymphadenopathy is common in both, children experience more fever and rash, and less generalized lymphadenopathy.
Area of Science:
- Pediatric Rheumatology
- Immunology
- Dermatology
Background:
- Kikuchi-Fujimoto disease (KFD) is a rare, benign, self-limiting condition.
- KFD is characterized by cervical lymphadenopathy and often affects young adults.
- Increasing recognition of KFD in pediatric populations necessitates understanding its unique characteristics.
Purpose of the Study:
- To delineate the distinct clinical features of KFD in pediatric patients.
- To compare the presentation and outcomes of KFD in children versus adults.
- To analyze epidemiological data of pediatric KFD cases.
Main Methods:
- Retrospective review of medical records for pediatric KFD patients (<18 years) from 2001-2012.
- Inclusion of worldwide published case reports of KFD.
- Comparative analysis of 140 pediatric cases against 733 cases of all ages.
Main Results:
- KFD in children shows a male predominance (1.4:1), unlike adult female predominance (2:1).
- Pediatric KFD commonly features tender cervical lymphadenopathy, fever (82%), and rash (10%), which are more frequent than in adults.
- Generalized lymphadenopathy is less common in children (1%) compared to adults (8%).
- Leukopenia was observed in 50% of children versus 38% of adults.
- Recurrence rates and autoimmune disease associations were similar between pediatric and adult KFD patients.
Conclusions:
- Cervical lymphadenopathy remains the hallmark of KFD across all age groups.
- Pediatric KFD exhibits a distinct clinical profile with higher incidences of fever and rash.
- Understanding these pediatric-specific features aids in accurate diagnosis and management of KFD in children.
Abstract:
Kikuchi-Fujimoto disease (KFD) is a benign, self-limiting disease characterized by cervical lymphadenopathy. Although it was primarily thought to be a disease of young adults, it has been increasingly recognized in children. To define the characteristics of KFD in children, we reviewed the medical records of patients younger than 18 years of age who were diagnosed with KFD from 2001 to 2012 at Korea University Medical Center, as well as worldwide published reports of KFD. A total of 140 pediatric patients and 733 patients of all ages was analyzed. Compared to the female predominance found in adults (2:1), young boys were more commonly affected than young girls (1.4:1). Cervical lymphadenopathy was the most common clinical finding in children, as it was in adults. Lymphadenopathy was more likely to be tender (69 vs. 44 %, p < 0.001) but less generalized (1 vs. 8 %, p < 0.05) in children compared to adults. Fever (82 vs. 35 %, p < 0.001) and rash (10 vs. 4 %, p < 0.05) were observed in children more commonly than in adults. Leukopenia was observed in 50 and 38 % of children and adults, respectively. Rates of recurrence and association with autoimmune diseases in children were comparable to those of adults. Cervical lymphadenopathy was the most common clinical manifestation of KFD in all ages. While fever and rash were more common in children with KFD compared to adults, generalized lymphadenopathy was rarer.
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