A Rare Cause of Recurrent Apthous Stomatitis in a Adult Patient and an Overlooked Entity: PFAPA Syndrome

Turk J Dermatol 2013;7(1):32-34

1. Suleyman Demirel University Medical Faculty, Department Of Skin And Venereal Diseases, Isparta, Turkey

2. Suleyman Demirel University Medical Faculty, Department Of Otolaryngology, Isparta, Turkey

3. Suleyman Demirel University School Of Medicine, Department Of Rheumatology, Department Of Internal Medicine, Isparta, Turkey

4. Suleyman Demirel University School Of Medicine, Department Of Internal Medicine, Department Of Immunology, Isparta, Turkey

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ABSTRACT

PFAPA syndrome is a recently identified clinical entity of unknown etiology characterized by recurrent episodes of abrupt onset of high fever, aphthous stomatitis, pharyngitis and cervical lymphadenopathy. The associated symptoms do not regress with antibiotic therapy. The dramatic response of symptoms to single dose corticosteroids is hallmark feature of this syndrome.

PFAPA syndrome usually begins under 5 years of age and in most cases resolves spontaneously until ten years old. PFAPA syndrome is extremely rare in adults, and to our knowledge, only about 20 cases have been described to date. Although PFAPA syndrome is increasingly reported in the pediatric literature, and is a known condition for the pediatricians, most dermatologists are unfamiliar with this entity.

In this report, we describe a late onset of PFAPA syndrome in a 25-year-old boy who presented to our clinic for his recurrent aphthous stomatitis and fever.