Department of Oral and Maxillofacial Medicine, School of Dentistry, Yazd Shahid Sadoughi University of Medical Sciences and Health Services, Yazd, Iran.
Department of Oral and Maxillofacial Surgery, School of Dentistry, Yazd Shahid Sadoughi University of Medical Sciences and Health Services, Yazd, Iran.
BMC Pediatr. 2023 Oct 13;23(1):506. doi: 10.1186/s12887-023-04344-z.
Zimmermann-Laband Syndrome (ZLS) and infantile systemic hyalinosis (ISH) are rare genetic disorders. They are characterized by various spectrum manifestations. In spite of other case reports, this case with features of both syndromes was reported by oral medicine specialists and oral and maxillofacial surgeons.
In this study, we reported an 18-months old female patient with gingival overgrowth. This phenomenon completely embedded all the erupted teeth. In this case, the presence of multiple papulonodular cutaneous lesions is a newly observed aspect that has rarely been reported in the existing literature. Gingival overgrowth was excised under general anesthesia. At six months of follow-up after surgery, mastication and breathing problems were improved. Aesthetic aspects were ameliorated in terms of gingival appearance.
To date, due to the ambiguous presentations, both syndromes remain an enigma for specialists. A timely diagnosis could be crucial for prognosis and preventing severe further surcharge. Dentists could play an important role in the diagnosis of rare disorders.
齐默尔曼-兰伯特综合征（ZLS）和婴儿系统性玻璃样变性（ISH）是罕见的遗传性疾病。它们的特征是具有各种不同的表现。尽管有其他病例报告，但口腔医学专家和口腔颌面外科医生报告了这例同时具有两种综合征特征的病例。
在本研究中，我们报告了一名 18 个月大的女性患者，其牙龈增生。这种现象完全覆盖了所有已萌出的牙齿。在该病例中，多发性丘疹结节性皮肤损害是一个新观察到的方面，在现有文献中很少有报道。在全身麻醉下切除了牙龈增生。术后 6 个月随访时，咀嚼和呼吸问题得到改善。在牙龈外观方面，美观方面得到了改善。
迄今为止，由于表现不明确，这两种综合征仍然是专家们的一个谜。及时诊断对于预后和防止严重的进一步加重至关重要。牙医在诊断罕见疾病方面可以发挥重要作用。
