Kshirsagar Jaishree Tukaram, Dharani Kalaiselvan, Thangavel Priyangha
Department of Periodontology, Tamil Nadu Government Dental College and Hospital, Chennai, Tamil Nadu, India.
J Indian Soc Periodontol. 2023 Nov-Dec;27(6):645-650. doi: 10.4103/jisp.jisp_582_22. Epub 2024 Jan 24.
Hereditary gingival fibromatosis (HGF) is an uncommon slow-growing fibrous overgrowth characterized by connective tissue accumulation. It presents as an isolated feature or as a manifestation of any syndrome. Various syndromes associated with HGF are inherited by autosomal dominant/recessive/X-linked traits. Zimmermann-Laband syndrome (ZLS) is a rare, autosomal dominant inherited disease manifested with gingival fibromatosis (GF), nose and ears abnormalities, and hypoplastic/dysplastic nails or terminal phalanges of hand and feet. Although the pattern of inheritance was found to be both autosomal dominant and recessive traits, the molecular basis is still unclear. This report presents a possible case of ZLS-associated HGF in a 25-year-old female patient who presents with GF, hypertrichosis, and other syndrome-related features. Her father was similarly affected whereas her mother and sibling were asymptomatic. The patient and her family members were explained about the condition and surgical periodontal therapy was carried out for the patient to improve esthetics and was followed up regularly. Esthetics was significantly improved and no recurrence was noted at the end of 6 months.
遗传性牙龈纤维瘤病（HGF）是一种罕见的、生长缓慢的纤维组织过度增生疾病，其特征为结缔组织堆积。它可表现为一种孤立的症状，也可作为任何综合征的一种表现形式。与HGF相关的各种综合征通过常染色体显性/隐性/X连锁性状遗传。齐默尔曼-拉班德综合征（ZLS）是一种罕见的常染色体显性遗传病，表现为牙龈纤维瘤病（GF）、鼻和耳异常，以及手和脚的指甲或指骨发育不全/发育异常。尽管已发现其遗传模式为常染色体显性和隐性性状，但分子基础仍不清楚。本报告介绍了一名25岁女性患者可能患有与ZLS相关的HGF的病例，该患者表现为GF、多毛症及其他与综合征相关的特征。她的父亲有类似症状，而她的母亲和兄弟姐妹无症状。向患者及其家庭成员解释了病情，并对患者进行了牙周手术治疗以改善美观，并定期进行随访。6个月末时美观得到显著改善，且未观察到复发情况。
