Abstract
Zimmerman-Laband syndrome was reported by Zimmerman in the year 1928 which is a rare inherited autosomal dominant disease characterized by generalized enlargement of the attached and marginal gingiva, abnormalities of nose, ear, deformities of nails, joint hyperextensibility, hepatosplenomegaly, skeletal abnormalities and occasional mental retardation. Idiopathic gingival enlargement is usually evident after the eruption of the permanent teeth. Both sexes are equally affected. Genetic loci for autosomal dominant modes of gingival fibromatosis is localized to chromosome 2p21p22 (HGF-1) and chromosome 5q12-q22 (HGF-2). This syndrome is not a life threatening disorder. Hereditary gingival enlargement is associated with syndromes like Rutherford syndrome, Zimmerman-Laband syndrome, Murray-Puretic-Drescher syndrome, Cross syndrome and Ramon’s syndrome. The most important feature of this syndrome is gingival enlargement appearing early in childhood. Idiopathic gingival enlargement is usually evident after the eruption of the permanent teeth. Surgical correction of gingival fibromatosis is recommended, although there is no information on the permanence of the results of this treatment. We present a case of a 14 year old female patient with Zimmerman-Laband syndrome. Gingivectomy was carried out in the upper and lower anterior region there by exposing the impacted teeth.
Highlights
Hereditary gingival enlargement is associated with syndromes like Rutherford syndrome, Zimmerman-Laband syndrome, MurrayPuretic-Drescher syndrome, Cross syndrome and Ramon’s syndrome [1]
Zimmerman-Laband syndrome was reported by Zimmerman in the year 1928 which is a rare inherited autosomal dominant disease characterized by generalized enlargement of the attached and marginal gingiva, abnormalities of nose, ear, deformities of nails, joint hyperextensibility, hepatosplenomegaly, skeletal abnormalities and occasional mental retardation
Idiopathic gingival enlargement is usually evident after the eruption of the permanent teeth
Summary
Hereditary gingival enlargement is associated with syndromes like Rutherford syndrome, Zimmerman-Laband syndrome, MurrayPuretic-Drescher syndrome, Cross syndrome and Ramon’s syndrome [1]. Zimmerman-Laband syndrome is a rare entity characterized by generalized enlargement of the attached and marginal gingiva, abnormalities of nose, ear and deformities of nails, joint hyperextensibility, hepatosplenomegaly, skeletal abnormalities and occasional mental retardation [2]. The prevalence of this syndrome is 1 in 10000000. Gingival fibromatosis is characterized by slow and progressive enlargement of maxillary and mandibular gingiva It can be presented as an isolated feature or as a part of syndrome. History revealed that her elder brother is suffering with the same condition hepatic fibrosis with portal hypertension Her parents were normal with no features of this syndrome. Patient is on regular follow up for assessing the recurrence
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