From Volume 38, Issue 2, March 2011 | Pages 106-110
Benign familial neutropenia (BFN) is a condition where there is a decrease in circulating neutrophils in the blood and patients suffer from oral manifestations which include: persistant periodontal disease, recurrent neutropenic ulceration and candidal infections. This report discusses a family affected by BFN and the effects on their oral health.
The different types of neutropenia can be divided up into the following groups:
The incidence of BFN is thought to be around 3-4 cases per million per year. The incidence of drug-induced neutropenia for comparison is about one case per million per year.1
Neutropenias are defined by an acute or chronic decrease in the peripheral blood neutrophil count, resulting in the individual being more susceptible to opportunistic infections. These may not last long in nature, when treated early and controlled by the appropriate antimicrobial or antifungal therapy, however, if the neutropenia is profound and there is a delay in the acute management of these infective episodes, then the patient faces a possible life-threatening situation.
The oral manifestations of neutropenias include:
The mucosal tissues are affected by characteristic neutropenic ulcers on the tongue and lips and cervical lymphadenopathy secondary to the ulceration (Figure 2). The neutropenic gingivitis is seen in its earliest manifestation in children affected by BFN (Figures 3 and 4). There can also be systemic manifestations which can include recurrent chest infections and skin infections. These manifestations can be seen either together or independently of each other.2
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