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References

Porter SR, Scully C, Oakhill A. Oral features of a family with benign familial neutropenia. J Am Acad Dermatol. 1994; 30:877-880
da Fonseca MA, Fontes F. Early tooth loss due to cyclic neutropenia: long-term follow up of one patient. Spec Care Dent. 2000; 20:(5)187-190
Shoenfeld Y, Weingerger A, Avishar R Familial leukopenia among Yemenite Jews. Isr J Med Sci. 1978; 14:1271-1274
Hakki SS, Aprikyan AA, Yildirim S, Aydinbelge M, Gokalp A, Ucar C Periodontal status in two siblings with severe congenital neutropenia: diagnosis and mutational analysis of the cases. J Periodont. 2005; 76:837-844
Busch FH. Familial benign chronic neutropenia in a Danish family. Ugeskr Laeger. 1990; 152:(36)2565-2566
Katsuragi Y, Matsuda N, Nakamura M, Murayama Y. Neutrophil functions in patients with severe periodontal disease. Adv Dent Res. 1998; 2:(2)359-363
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Kirstila V, Sewon L, Laine J. Periodontal disease in three siblings with familial neutropenia. J Periodont. 1993; 64:(6)566-570
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Case report of a family with benign familial neutropenia and the implications for the general dental practitioner

From Volume 38, Issue 2, March 2011 | Pages 106-110

Authors

Christine Casey

Senior House Officer in Child Dental Health, Department of Child Dental Health, Bristol Dental Hospital, Lower Maudlin Street, Bristol, BS1 2LY

Articles by Christine Casey

Tony Brooke

Associate Specialist, Department of Oral Medicine, Bristol Dental Hospital, Lower Maudlin Street, Bristol BS1 2LY, UK

Articles by Tony Brooke

Rebecca Davies

BChD, MFDS RCS, MSc DDR

SpR Dental and Maxillofacial Radiology, Bristol and Birmingham

Articles by Rebecca Davies

Deborah Franklin

Consultant in Paediatric Dentistry, Department of Child Dental Health, Bristol Dental Hospital, Lower Maudlin Street, Bristol BS1 2LY, UK

Articles by Deborah Franklin

Abstract

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.

Clinical Relevance: Benign familial neutropenia is a rare condition and this article aims to raise awareness among general dental practitioners so that prompt referral and management in secondary care can be arranged.

Article

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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