Papillon-Lefèvre syndrome: Case report of 3 siblings in consanguineous family

Ayesha Anwar, Wasifa Hayat, Shahbaz Aman

Abstract


The Papillon-Lefèvre syndrome (PLS) is a very rare syndrome of autosomal recessive inheritance characterized by palmoplantar hyperkeratosis and early onset periodontitis, leading to premature loss of both primary and permanent dentition. We report three cases of PLS in the same family who presented with all characteristic features. All patients presented with persistent thickening, flaking and scaling of the skin of palms and soles, mobility and rapid loss of teeth. Severe gingival inflammation, abscess formation, and deep periodontal pockets along with loss of teeth were evident on intraoral examination.

 


Keywords


Palmoplantar hyperkeratosis, Papillon-Lefèvre syndrome, periodontitis

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References


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