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Measles Lymphadenopathy in a Child With PFAPA Syndrome
Jefferson Terry1, Kelly Brown2, Joanne Hiebert3
11 Department of Pathology and Laboratory Medicine, University of British Columbia and British Columbia Children's Hospital, Vancouver, British Columbia, Canada.
Insights
Measles virus was detected in the tonsils of a child with Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) syndrome. This finding may offer insights into the immune dysregulation associated with PFAPA.
Area of Science:
- Pediatrics
- Immunology
- Virology
Background:
- Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) syndrome is a common pediatric autoinflammatory disorder.
- Its exact cause is unknown, but immune dysregulation and environmental factors are suspected.
- Tonsillectomy is an effective treatment for some patients, yet the underlying mechanisms remain unclear.
Observation:
- Histological examination of tonsils from a PFAPA patient revealed measles-type viral cytopathic effects.
- Molecular analysis detected measles virus RNA in tonsillar tissue.
- Further testing suggested the presence of measles vaccine strain RNA, though confirmation was limited.
Findings:
- Measles virus RNA was identified in the tonsil of a child diagnosed with PFAPA syndrome.
- The presence of viral RNA in tonsillar tissue warrants further investigation.
- This case presents a potential link between measles virus and PFAPA.
Implications:
- The findings suggest a possible role for measles virus in PFAPA pathogenesis or immune response.
- Further research is needed to explore the association between measles virus and PFAPA.
- Understanding this connection could lead to new diagnostic or therapeutic strategies for PFAPA.
Abstract:
Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is a common cause of periodic fever in children. The pathogenesis of PFAPA is unknown but likely involves immune system dysregulation and may be initiated by an environmental trigger. Tonsillectomy resolves or improves symptoms in some patients, but the reason for this is unknown; moreover, specific abnormalities in tonsillectomy specimens from PFAPA patients have not been described. Here, we report measles virus in tonsil from a child with PFAPA. Measles-type viral cytopathic effect was discovered on histological examination of tonsillar tissue after therapeutic tonsillectomy for PFAPA. Molecular testing showed the left tonsil was positive for measles RNA by reverse transcription polymerase chain reaction (RT-PCR) while the right tonsil was inconclusive (weakly positive). Real-time RT-PCR specific for measles vaccine strain RNA (genotype A) was weakly reactive in the left tonsil tissue when tested in 3 independent replicates, but this result could not be confirmed with conventional genotyping by sequencing. The relationship and clinical significance between measles virus and PFAPA in this case is unclear but may be related to PFAPA-associated immune dysregulation. Additional investigation of measles virus in PFAPA patients would be helpful in further exploring this potential association.
