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Zimmermann-Laband syndrome and infantile systemic hyalinosis: an enigma with two separate terms with overlapping features: a case report
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Zimmermann-Laband syndrome and infantile systemic hyalinosis: an enigma with two separate terms with overlapping features: a case report
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Journal Article
Zimmermann-Laband syndrome and infantile systemic hyalinosis: an enigma with two separate terms with overlapping features: a case report
2023
Overview
Background
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.
Case presentation
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.
Conclusions
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.
Publisher
BioMed Central,BioMed Central Ltd,Springer Nature B.V,BMC
Subject
Abnormalities, Multiple - genetics
/ Babies
/ Biopsy
/ Craniofacial Abnormalities - diagnosis
/ Diarrhea
/ Female
/ Fibromatosis, Gingival - diagnosis
/ Fibromatosis, Gingival - genetics
/ Fibromatosis, Gingival - pathology
/ Humans
/ Hyaline Fibromatosis Syndrome
/ Infant
/ Infantile systemic hyalinosis
/ Medicine
/ Neck
/ Skin
/ Teeth
