Nonsurgical periodontal management of Papillon–Lefevre syndrome

• Published in: Journal of interdisciplinary dentistry 2021-05, Vol.11 (2), p.101-105
• Main Authors: Guruprasad, C, Shankareswari, T, Jeethu, Maria, Basavarajappa, Suman
• Format: Article
• Language: English
• Subjects: Autosomal recessive inheritance; Bone loss; Consanguinity; Dentition; Diagnosis; Dipeptidyl-peptidase I; Genetic disorders; Gum disease; Hereditary diseases; Keratosis; Oral cavity; Patients; Periodontal diseases; Skin diseases; Skin lesions; Teeth
• ISSN: 2229-5194; 2231-2706
• DOI: 10.4103/jid.jid_67_20

Papillon–Lefevre syndrome (PLS) is an autosomal recessive genetic disorder caused by a deficiency in Cathepsin C. It is characterized by palmoplantar keratosis and premature loss of both primary and permanent dentition. Patients are often edentulous at an early age. Other symptoms include increased susceptibility to infections. Various etiologic factors such as genetic mutations, immunologic alterations, and bacteria have been implicated. We present a case report of 12-year-old female who reported to the dental clinic with a history of pain and swollen gums. Family history revealed consanguinity. On clinical examination, there was palmoplantar keratosis and examination of the oral cavity showed generalized periodontal pockets, tooth mobility, and periodontal abscess. Radiographic examination revealed generalized horizontal bone loss. Lateral cephalogram revealed retarded somatic development. A diagnosis of PLS was arrived based on history, clinical and radiological findings. Nonsurgical periodontal treatment along with the treatment of skin lesions was performed. A combined and intensive mechanical and antimicrobial treatment with supportive periodontal treatment in PLS patients may halt the periodontal disease progression. The dentists should be aware of the same because an early diagnosis of the syndrome can help to preserve the teeth by the timely institution of treatment, using a multidisciplinary approach.