Zimmermann Laband Syndrome: A Rare Case Report

Case Report

J Dent & Oral Disord. 2022; 8(1): 1174.

Zimmermann Laband Syndrome: A Rare Case Report

Afsar A1*, Gupta R2, Dahiya P3 and Gupta M4

1MDS 3rd Year Student, Department of Periodontics, Himachal Institute of Dental Sciences, HP, India

2MDS, Professor and Head of Department of Periodontics, Himachal Institute of Dental Sciences, HP, India

3MDS, Professor, Himachal Institute of Dental Sciences HP, India

4MDS, Professor, DAV Dental College, Yamunanagar, India

*Corresponding author: Afsar A, MDS 3rd Year Student, Department of Periodontics, Himachal Institute of Dental Sciences, HP, India

Received: June 08, 2022; Accepted: July 05, 2022; Published: July 12, 2022


The Zimmermann Laband Syndrome was first described by Zimmermann in the year 1928. It is an extremely rare autosomal dominant congenital disorder. It is mainly characterized by gingival fibromatosis, multiple unerupted teeth, coarse facial appearance, hypertrichosis and sometimes hepatosplenomegaly. The characterstics are highly variable. The present case report is of a 14 year old boy who was diagnosed as Zimmermann Laband Syndrome based on the classical sign of gingival overgrowth.

Keywords: Zimmermann; Hypertrichosis; Hyperplasia; Enlargement; Dentistry


Gingival enlargement is the overgrowth of the gingiva characterized by an expansion and accumulation of the connective tissue with occasional presence of increased number of cells. It is caused by several factors, such as inflammation, leukemia, drugs, and inheritance. The inheritance condition in which the gingival tissue spontaneously and progressively enlarges is identified as hereditary gingival fibromatosis (HGF) [1].

Zimmermann-Laband Syndrome (ZLS) is a very rare condition characterized by gingival fibromatosis, coarse facial appearance and malformed nails or distal phalanges of hands/feet and sometimes hepatosplenomegaly. The term Zimmermann-Laband Syndrome was for the first time given by Carl Jacob Witkop [2]. It was found that there is mutations in KCNH1 and ATP6V1B2 occurs which causes Zimmermann-Laband syndrome.

Most commonly it is associated with local or diffuse nonhemorrhagic gingival enlargement. The underlying alveolar bone is not directly influenced by gingival hyperplasia, but the gingival excess can allow plaque accumulation, causing biofilm-induced gingivitis, pseudo pockets, caries, and halitosis and there is failure of deciduous and permanent tooth eruption often occurs [3]. The most common extra oral clinical features present in this condition are bulbous soft nose, thick floppy ears, nail aplasia or hypoplasia, hypertrichosis, joint hyperextensibility, hepatosplenomegaly, and intellectual disability with or without epilepsy [4].

Case Report

A 14-year-old male patient was reported to the hospital with the chief complaint of increase in thickness of the gums and no visibility of teeth in the upper and lower anterior region causing difficulty in chewing and also an unpleasant smile. During history examination it was revealed that the increase in size starts 1 year ago had reached the present size. There was no familial history elicited during examination. On general examination the child was systemically healthy and was not on any medication.

On intraoral examination of patient it was observed that there was thick enlargement of maxillary and mandibular gingiva and all the four quadrants were involved. There was minimal bleeding on probing was present and the teeth present were partially or fully covered with gingival overgrowth. Also there were multiple carious teeth and presence of plaque and calculus. During extra oral observation there was increase in the size of the lips which gives prominent labial fullness. Patient’s nose was bulbous and ears were folded including the feature of hypertrichosis as shown in (Figure I and II).