A Case of Adult-Onset Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome Responsive to Tonsillectomy in Japan
Adult-onset periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is a rare condition, having been reported in only three patients in Japan till date. While almost all pediatric PFAPA patients respond well to tonsillectomy, some European studies have reported that...
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| Format: | Article |
| Language: | English |
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Wiley
2019-01-01
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| Series: | Case Reports in Otolaryngology |
| Online Access: | http://dx.doi.org/10.1155/2019/1746180 |
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| author | Kohei Yamahara Yuki Egawa Kana Lee Noriyuki Nakashima Satoshi Ikegami |
| author_facet | Kohei Yamahara Yuki Egawa Kana Lee Noriyuki Nakashima Satoshi Ikegami |
| author_sort | Kohei Yamahara |
| collection | DOAJ |
| description | Adult-onset periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is a rare condition, having been reported in only three patients in Japan till date. While almost all pediatric PFAPA patients respond well to tonsillectomy, some European studies have reported that tonsillectomy may be ineffective for adult-onset PFAPA. All the Japanese patients with adult-onset PFAPA had been treated orally so far (cimetidine with or without prednisone), instead of tonsillectomy. We reported a case involving a 37-year-old Japanese man with PFAPA syndrome who presented with a history of febrile episodes associated with pharyngitis, cervical adenitis, and aphthous stomatitis for one year. The patient had been undergoing oral medication therapy without any significant improvement. Tonsillectomy was performed for the patient, and complete resolution of PFAPA was achieved. Our experience suggests that a tonsillectomy is a viable option for the treatment of adult-onset PFAPA. |
| format | Article |
| id | doaj-art-6c27b7fb3cea44f99606b594afca7f7e |
| institution | Kabale University |
| issn | 2090-6765 2090-6773 |
| language | English |
| publishDate | 2019-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Otolaryngology |
| spelling | doaj-art-6c27b7fb3cea44f99606b594afca7f7e2025-08-20T03:33:45ZengWileyCase Reports in Otolaryngology2090-67652090-67732019-01-01201910.1155/2019/17461801746180A Case of Adult-Onset Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome Responsive to Tonsillectomy in JapanKohei Yamahara0Yuki Egawa1Kana Lee2Noriyuki Nakashima3Satoshi Ikegami4Department of Otolaryngology, Head and Neck Surgery, Shizuoka City Shizuoka Hospital, Shizuoka, Shizuoka 420-8630, JapanDepartment of Pathology, Shizuoka City Shizuoka Hospital, Shizuoka, Shizuoka 420-8630, JapanDepartment of Otolaryngology, Shin-Suma General Hospital, Kobe, Hyogo 654-0048, JapanDepartment of Physiology, Kurume University School of Medicine, 67 Asahi-Machi, Kurume 830-0011, JapanDepartment of Otolaryngology, Head and Neck Surgery, Shizuoka City Shizuoka Hospital, Shizuoka, Shizuoka 420-8630, JapanAdult-onset periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is a rare condition, having been reported in only three patients in Japan till date. While almost all pediatric PFAPA patients respond well to tonsillectomy, some European studies have reported that tonsillectomy may be ineffective for adult-onset PFAPA. All the Japanese patients with adult-onset PFAPA had been treated orally so far (cimetidine with or without prednisone), instead of tonsillectomy. We reported a case involving a 37-year-old Japanese man with PFAPA syndrome who presented with a history of febrile episodes associated with pharyngitis, cervical adenitis, and aphthous stomatitis for one year. The patient had been undergoing oral medication therapy without any significant improvement. Tonsillectomy was performed for the patient, and complete resolution of PFAPA was achieved. Our experience suggests that a tonsillectomy is a viable option for the treatment of adult-onset PFAPA.http://dx.doi.org/10.1155/2019/1746180 |
| spellingShingle | Kohei Yamahara Yuki Egawa Kana Lee Noriyuki Nakashima Satoshi Ikegami A Case of Adult-Onset Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome Responsive to Tonsillectomy in Japan Case Reports in Otolaryngology |
| title | A Case of Adult-Onset Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome Responsive to Tonsillectomy in Japan |
| title_full | A Case of Adult-Onset Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome Responsive to Tonsillectomy in Japan |
| title_fullStr | A Case of Adult-Onset Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome Responsive to Tonsillectomy in Japan |
| title_full_unstemmed | A Case of Adult-Onset Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome Responsive to Tonsillectomy in Japan |
| title_short | A Case of Adult-Onset Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome Responsive to Tonsillectomy in Japan |
| title_sort | case of adult onset periodic fever aphthous stomatitis pharyngitis and cervical adenitis pfapa syndrome responsive to tonsillectomy in japan |
| url | http://dx.doi.org/10.1155/2019/1746180 |
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