Periodontal Manifestations of Chronic Atypical Neutrophilic Dermatosis With Lipodystrophy and Elevated Temperature (CANDLE) Syndrome in an 11-Year-Old Patient.

Abstract

Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) is an autoinflammatory syndrome caused by an autosomal recessive gene mutation. This very rare syndrome has been reported in only 14 patients worldwide. A number of clinical signs have been reported, including joint contractures, muscle atrophy, microcytic anemia, and panniculitis-induced childhood lipodystrophy. Additional symptoms include recurrent fevers, purpuric skin lesions, periorbital erythema, and failure to thrive. To the best of the authors' knowledge, this is the first reported case of periodontal manifestations associated with CANDLE syndrome. An 11-year-old boy was referred to Cork University Dental School and Hospital, Cork, Ireland, with evidence of severe periodontal breakdown. The patient's medical condition was managed in Great Ormond Street Children's Hospital, London, United Kingdom. The patient's dental management included initial treatment to remove teeth of hopeless prognosis, followed by prosthodontic rehabilitation using removable partial dentures. This was followed by additional non-surgical periodontal treatment and maintenance. In the long term, the potential definitive restorative options, including dental implants, will be evaluated in discussion with the patient's medical team. Periodontitis as a manifestation of systemic disease is one of seven categories of periodontitis as defined by the American Academy of Periodontology 1999 classification system. A number of systemic diseases have been associated with advanced periodontal breakdown, including diabetes mellitus, leukemia, and Papillon-Lefèvre syndrome. In the case described, treatment necessitated a multidisciplinary approach with input from medical and dental specialties for a young patient with severe periodontal breakdown associated with CANDLE syndrome.