Percorsi clinici
Una PFAPA che diventa un Behçet
A PFAPA that becomes BeHçet’s disease
Claudia Cafolla1,2, Alessandra Iacono1, Claudia Guiducci1, Lorenzo Mambelli1, Federico Marchetti1
1UOC di Pediatria e Neonatologia Ospedale di Ravenna, AUSL della Romagna
2Scuola di Specializzazione in Pediatria, Università di Ferrara
Aprile 2021 - pagg. 226 -229 | DOI: 10.53126/MEB40226
Abstract
PFAPA syndrome (periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis) is the most common periodic fever syndrome in children. The disease appears to cluster in families, but the pathogenesis is unknown. The paper reports the description of a clinical case of a 12-year-old girl diagnosed with PFAPA syndrome from the age of one and half years. The exacerbations did not decrease with growth as it usually happens, but genital ulcers appeared associated with HLA-B51 positivity, orienting the diagnosis towards Behçet’s spectrum disorders.
Riassunto
La febbre periodica, stomatite aftosa, faringite e adenite cervicale (PFAPA) è la sindrome da febbre periodica più comune nei bambini. La malattia sembra avere in alcuni casi una base familiare, ma la patogenesi è sconosciuta. Riportiamo il caso di una paziente di 12 anni con esordio di PFAPA all’età di 1 anno e mezzo. Le riacutizzazioni della patologia non si sono ridotte con la crescita come normalmente accade, al contrario sono comparse delle ulcere genitali in associazione ad una positi-vità per HLA-B51 che ci hanno portato ad orientare la diagnosi verso una nuova entità clinica che viene chiamata disturbo dello spettro della malattia di Behçet.
Parole chiave
Classificazione MeSH
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Bibliografia
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Corrispondenza: alessandra.iacono@auslromagna.it