| A case of periodic fever, aphthous ulcers, pharyngitis,and cervical adenitis syndrome in an adult |
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Sang Ock Kim, Chien Ter Hsing, Ji Sun Han, Byung Geun Kim, Ha Yeon Kim, Won Tae Chung, Sung Won Lee |
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| 성인에서 진단된 PFAPA증후군 1예 |
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김상옥·형건덕·한지선·김병근·김하연·정원태·이성원, Chien Ter Hsing, Ji Sun Han, Byung Geun Kim, Ha Yeon Kim, Won Tae Chung, Sung Won Lee |
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| Abstract |
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In 1987, Marshall et al. described a new periodic fever that was later named periodic fever, aphthous ulcers, pharyngitis, and cervical
adenitis (PFAPA) syndrome. Other periodic fever diseases, such as recurrent tonsillitis, a number of infectious diseases, juvenile
idiopathic arthritis, Behcet’s disease, cyclic neutropenia, familial Mediterranean fever (FMF), familial Hibernian fever (FHF),
and hyperglobulinema D syndrome must be excluded as diagnoses. The diagnostic criteria were proposed in 1989 and modified 10
years later. Adult onset PFAPA syndrome has rarely been reported and has not been reported in Korea. We diagnosed PFAPA syndrome
with characteristic clinical findings in a 50-year-old man, who showed good response to steroid therapy. (Korean J Med
77:S1332-S1336, 2009) |
| Key Words:
PFAPA syndrome; Marshall’s syndrome; Periodic disease; Rare disease |
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