Keele University Medical School, Staffordshire, England, UK.
Indian J Pediatr. 2013 May;80(5):425-7. doi: 10.1007/s12098-012-0744-6. Epub 2012 Apr 13.
PFAPA (periodic fever, apthous stomatitis, pharyngitis, cervical adenitis) is a rare condition of unknown cause affecting children. Although the exact etiology is unknown, inflammatory, immunological or genetic causes have been suggested. The diagnosis is made by exclusion of other causes of periodic fever. Although management is essentially symptomatic, single corticosteroid dose, tonsillectomy and Cimetidine has been shown to be associated with resolution of symptoms. Although abdominal pain and genital ulcers have been reported in association with PFAPA, unilateral transient facial swelling has not been previously reported. The authors present a hitherto unreported association of PFAPA with recurrent episodes of unilateral facial swelling.
PFAPA（周期性发热、口疮性口炎、咽炎、颈淋巴结炎）是一种罕见的病因不明的疾病，影响儿童。尽管确切的病因尚不清楚，但已提出炎症、免疫或遗传原因。通过排除其他周期性发热的原因来做出诊断。尽管治疗主要是对症治疗，但已证明单剂量皮质类固醇、扁桃体切除术和西咪替丁与症状缓解有关。尽管已经报道 PFAPA 与腹痛和生殖器溃疡有关，但以前没有报道过单侧短暂性面部肿胀。作者提出了 PFAPA 与反复发作的单侧面部肿胀之间以前未报道过的关联。
