Cyclic neutropenia presenting as recurrent oral ulcers and periodontitis

Cyclic neutropenia (CN) is a rare congenital disease that can present with recurrent oral ulcers and periodontitis. CN can easily be misdiagnosed as major recurrent aphthous stomatitis (MaRAS) or aggressive periodontitis (AP) in dental clinics. We describe the case of an 8-year-old boy with CN, and compare the oral manifestations of CN with those of MaRAS and AP.

An 8-year-old boy presented with a history of recurrent oral ulcers, periodontal destruction, pharyngitis and otitis media since the age of 3 months. Repeated, routine blood tests showed 1-week-long neutropenic periods that occurred at intervals of 2 weeks. A bone marrow cytology test during a neutropenic period demonstrated a decrease in granulocyte count. During a 2-year follow-up, his symptoms were well controlled by regular administration of granulocyte colony-stimulating factor and periodontal maintenance.

Several clinical features help to differentiate CN from MaRAS and AP Early recognition of the systemic cause of oral symptoms is important.