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  • 标题:Rhinophyma in tuberous sclerosis complex: case report with brief review of literature
  • 本地全文:下载
  • 作者:Reinhard E. Friedrich ; Christian Hagel
  • 期刊名称:GMS Interdisciplinary Plastic and Reconstructive Surgery DGPW
  • 印刷版ISSN:2193-8091
  • 电子版ISSN:2193-8091
  • 出版年度:2014
  • 卷号:3
  • DOI:10.3205/iprs000053
  • 语种:English
  • 出版社:German Medical Science
  • 摘要:

    Tuberous sclerosis or tuberous sclerosis complex (TSC) is an autosomal dominant inherited disease characterized by the triad epilepsy, hamartomas (angiofibroma) and reduced intellectual capacity. Phenotype can vary considerably. Almost all patients with TSC have at least one characteristic dermatologic feature. Facial angiofibroma can cause severe disfigurement. It may involve the cheeks, perioral region and nose, resulting in thick layers of nodular and pustular skin. Aesthetic surgery of the face comprises an individually adapted ablation of the affected skin regions in order to improve physical appearance. Reports on the subject of surgery for nasal angiofibroma confirm the homogenous transformation of the connective tissues by this hamartoma. Hitherto there is only one report in the literature describing the typical epithelial alterations of the nasal skin compatible with a rhinophyma and adjacent angiofibroma. Here we report the successful electrosurgical treatment of a patient with TSC and extensive sebaceous glands giving rise to a rhinophyma in close association with angiofibroma.

  • 关键词:tuberous sclerosis; rhinophyma; angiofibroma; adenoma sebaceum; rhinophyma surgery; rhinophyma surgical; disfigurement; electrosurgery; phacomatosis
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