Papillon Lefevre syndrome associated with recurrent pyogenic abscess of facial skin followed by scarring: A case report

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.