INTRODUCTION: Tuberous sclerosis (TS) is an autosomal dominant neuro-cutaneous syndrome characterized by multiple hamartomas in various organs, especially on skin and central nervous system. The most common features of TS include facial angiofibromas, hypomelanotic cutaneous macules, shagreen patches in the lumbar area, cerebral cortical tubers, sub-ependymal nodules, sub-ependymal giant cell astrocytomas, cardiac rhabdomyomas, and renal angiomyolipomas. Frequently oral manifestations such as fibrous hyperplasia, angiofibromas and dental enamel pitting are also observed. The aim of this case report was to describe the histological aspects of oral diffuse hyperplastic angiofibromatosis, never reported in the English literature and analyzed by Confocal Laser Scanning Microscope (CLSM), and to highlight the surgical implications of these aspects such as use of Diode Laser. CASE REPORT: A 14-years-old female patient with TS diagnosis came to our attention for diffuse gingival hyperplasia on the mandible. Clinical examination highlighted epidermal hamartomas on the whole body, especially on the face and scalp. Pathologic hyperplastic tissue was removed by pulsed diode laser at the power of 5-6W, and the surgical samples were sent for conventional and CLSM histopathological examination. After laser excision, wounds healed quickly without complications. At CLSM examination collagen fibres, showing intense fluorescence and with variable spatial orientation, and variably sized blood vessels were noticed suggesting the diagnosis of gingival angiofibromatosis, a still unreported finding in TS patients. CONCLUSIONS: CLSM analysis allows to highlight some unusual histopathological features of TS; diode laser is very effective for the treatment of gingival angiofibromatosis.
INTRODUCTION:Tuberous sclerosis (TS) is an autosomal dominant neuro-cutaneous syndrome characterized by multiple hamartomas in various organs, especially on skin and central nervous system. The most common features of TS include facial angiofibromas, hypomelanotic cutaneous macules, shagreen patches in the lumbar area, cerebral cortical tubers, sub-ependymal nodules, sub-ependymal giant cell astrocytomas, cardiac rhabdomyomas, and renal angiomyolipomas. Frequently oral manifestations such as fibrous hyperplasia, angiofibromas and dental enamel pitting are also observed. The aim of this case report was to describe the histological aspects of oral diffuse hyperplastic angiofibromatosis, never reported in the English literature and analyzed by Confocal Laser Scanning Microscope (CLSM), and to highlight the surgical implications of these aspects such as use of Diode Laser. CASE REPORT: A 14-years-old female patient with TS diagnosis came to our attention for diffuse gingival hyperplasia on the mandible. Clinical examination highlighted epidermal hamartomas on the whole body, especially on the face and scalp. Pathologic hyperplastic tissue was removed by pulsed diode laser at the power of 5-6W, and the surgical samples were sent for conventional and CLSM histopathological examination. After laser excision, wounds healed quickly without complications. At CLSM examination collagen fibres, showing intense fluorescence and with variable spatial orientation, and variably sized blood vessels were noticed suggesting the diagnosis of gingival angiofibromatosis, a still unreported finding in TS patients. CONCLUSIONS: CLSM analysis allows to highlight some unusual histopathological features of TS; diode laser is very effective for the treatment of gingival angiofibromatosis.
Authors: José-Francisco Millán-Cayetano; Oriol Yélamos; Anthony M Rossi; Michael A Marchetti; Manu Jain Journal: Dermatol Pract Concept Date: 2017-01-31