Tuberous Sclerosis Complex with Gingival Enlargement in an Adolescent.

JCPSP-JOURNAL OF THE COLLEGE OF PHYSICIANS AND SURGEONS PAKISTAN(2018)

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摘要
Tuberous sclerosis complex (TSC) is an autosomal dominant, multisystem genetic disorder. It is characterised by formation of benign hamartomas, neurofibromas, and angiofibromas located in different organs. We describe a case of a 13-year boy who complained of gingival enlargement. Clinical examination showed distinctive dermatological signs like hypopigmented macules, shagreen plaques, miliary fibromas, fibrous plaques and multiple angiofibromas. Oral manifestation included localised gingival enlargement. Gingivectomy was performed and the excised tissue was submitted for histopathological examination. The microscopic examination of gingival tissue revealed multiple bundles of collagen fibres with proliferating fibroblast and multiple proliferating blood vessels in the connective tissue. The clinical and histopathological findings were consistent with gingival angiofibromas of TSC. Gingivectomy allowed the patient to have better function and aesthetics. Periodontal examination in conjunction with dermatological examination is important for early diagnosis of TSC.
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关键词
Gingival enlargement,Gingivectomy,Neurocutaneous syndromes,Tuberous sclerosis
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