Jyoti Prajapat

Abstract

A familial non-syndromal supernumerary teeth is a rare condition encountered in dental practice. Supernumerary teeth occur in excess of the usual number of teeth. Multiple supernumerary teeth are associated with some syndromes like cleidocranial dyplasia and Gardner syndrome. This report describes the occurrence of supernumerary premolar teeth at the same location in two sisters as a non-syndromal trait. General dental practitioners (GDP) and dental specialists should have the knowledge and skills to diagnose, manage and treat patients with this condition safely and effectively. It will also discuss the prevalence, unique distribution, associated conditions, aetiology and treatment options of this condition. This paper describes the considerations a dental practitioners should take when they encounter a case of supernumerary teeth. Keywords: Supernumerary teeth, Premolars, Impacted teeth, Hyperdontia.

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Abstract

Papillon-Lefévre syndrome (PLS) is an autosomal recessive disorder that is characterised by severe generalized early-onset periodontitis and palmoplantar hyperkeratosis. Besides these cardinal features some patients rarely exhibits recurrent pyogenic infections of skin, hair follicle infection (frunculosis), multiple abscess of skin, hyperhidrosis. This article represents a rare case of 17 year old young male patient with cardinal features of PLS along with history of recurrent pyogenic abscess of facial skin followed by scarring since childhood. There are multiple etiological factors like genetic, immunological, and microbial factors, consanguineous marriage and reduced host defense mechanism. Patients with PLS have increased susceptibility to infections. Over 300 cases have been reported till now in dental and medical literature with most of the cases discovered amongst Africans, Arabs and, Indians with 25% patients showing recurrent pyogenic infections. Management of the disorder primarily involves conventional periodontal treatment; oral hygiene instructions, antiseptic mouth rinses; and systemic antibiotic. Skin lesions are usually treated with topical application of emollients, keratolytic agents, steroids, and oral retinoid. Keywords: PapillonLefevre syndrome, Early onset periodontitis, Cathepsin C, Palmoplantar hyperkeratosis, Premature exfoliation of teeth.

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