Papillon-Lefevre syndrome (PLS) is a condition characterized by dermatological manifestations and early onset periodontitis. The pathogenesis of PLS is secondary to mutation of the cathepsin C gene. Hence, the manifestations are expressed on the areas of the body covered by epithelium, such as palms, soles, knees and keratinized oral gingiva. Various immune cells, including polymorphonuclear leukocytes, macrophages, and their precursors are also affected leading to functional disability. PLS is an autosomal recessive condition and can occur in siblings born of consanguineous marriages. This report highlights a rare instance of two siblings of a family affected with Papillon-Lefevre syndrome.
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