Cunha John Lennon Silva, Ramos Maria Alice Carvalho da Cruz, Regis Débora Menezes, Sanchéz-Romero Celeste, de Andrade Maria Eliane, Bezerra Bruno Torres, de Albuquerque-Júnior Ricardo Luiz Cavalcanti
University of Campinas (UNICAMP), Piracicaba Dental School, Department of Oral Diagnosis. Piracicaba, SP, Brazil.
Tiradentes University, Department of Dentistry. Aracaju, SE, Brazil.
Autops Case Rep. 2020 Jan 21;10(1):e2020140. doi: 10.4322/acr.2020.140. eCollection 2020 Jan-Mar.
Hereditary gingival fibromatosis (HGF) is a rare genetic condition characterized by slow and progressive gingival enlargement. The gingival overgrowth often delays teeth eruption and may cause serious functional and aesthetic problems. We reported a case of a 10-year-old female child presenting a generalized gingival enlargement covering almost all the maxillary and mandibular teeth and resulted in problems for swallowing, speaking, and poor aesthetics. An incisional biopsy was performed and revealed a hypocellular and hypovascular dense collagenous tissue covered by squamous epithelium exhibiting acanthosis and elongated rete ridges. The diagnosis was HGF. The treatment instituted was an association of gingivectomy with a rigorous program of oral hygiene and follow-up. Herein, we describe a rare non-syndromic case of generalized HGF, including clinical and microscopical features, as well as highlighting the importance of correct diagnosis of this genetic condition.
遗传性牙龈纤维瘤病(HGF)是一种罕见的遗传性疾病,其特征为牙龈缓慢进行性增大。牙龈过度生长常常延迟牙齿萌出,并可能导致严重的功能和美观问题。我们报告了一例10岁女童病例,其表现为广泛性牙龈增大,几乎覆盖了所有上颌和下颌牙齿,导致吞咽、说话困难以及美观不佳。进行了切取活检,结果显示为一种细胞稀少、血管减少的致密胶原组织,表面覆盖有表现为棘层增厚和延长的 rete 嵴的鳞状上皮。诊断为HGF。所采取的治疗方法是牙龈切除术联合严格的口腔卫生计划及随访。在此,我们描述了一例罕见的非综合征性广泛性HGF病例,包括临床和显微镜特征,并强调了正确诊断这种遗传性疾病的重要性。