| Popis: |
CANDLE Syndrome (Chronic Atypical Neutrophilic Dermatosis with Lipodystrophy and Elevated Temperature) is an autoinflammatory disease. Clinical characteristics include recurrent fevers, organ inflammation, skin lesions, anemia, lipodystrophy, basal ganglion calcifications, and delayed physical development. Orofacial manifestations include facial lipodystrophy, swollen eyelids, thick lips, macroglossia, generalized microdontia, and jaw osteopenia. We describe a novel finding of multiple inflamed follicular cysts in two patients, diagnosed with CANDLE syndrome, who presented with radiolucent mandibular lesions associated with displaced permanent molars and mandibular bony expansion. To the best of our knowledge, the finding of inflamed follicular cysts in patients with CANDLE syndrome has never been reported in literature. We speculate that the intense inflammation seen in both cases could possibly corelate with the inflammatory dysregulation and generalized organ inflammation seen in this disease process. This new finding of radiolucent mandibular cystic lesions provides further insight into the extent and characterization of the syndrome. |
