Two cases of Papillon-Lefevre syndrome (PLS) in paediatric patients treated with a multidisciplinary approach have been identified in the current case series. Papillon-Lefevre syndrome (PLS) is an unusual heterogeneous autosomal recessive trait characterised by erythematous palmoplantar hyperkeratosis, periodontitis of early onset, and dura mater associated calcification. With genetic, immunological, and microbial factors playing a role in etiopathogenesis, the aetiology of PLS is multifactorial. The recently discovered PLS genetic defect was mapped to chromosome 11q14-q21, which includes cathepsin C mutations. This paper presents a report of 2 cases of Papillonlefevre syndrome in which diagnosis is based on clinical presentation and genetic mapping.PLS in an extremely rare condition is associated with lifelong psychological and social impacts on the growing children such as depression including hopelessness, aimlessness, social phobia, and a fear of communicating with people outside their family.
Author(s) Details
Dr. Priti A. Charde
Department of Periodontology and Implantology,
Sharad Pawar Dental College and Hospital, Wardha, Maharashtra 442001,India..
Dr. Shweta Kale
Department of Prosthodontics, Sharad Pawar Dental College and Hospital,
Wardha, Maharastra 442001, India.
Dr. M. L. Bhongade
Department of Periodontology and
Implantology, Sharad Pawar Dental College and Hospital, Wardha, Maharashtra
442001,India.
Dr. Priyanka Jaiswal
Dr. Aniruddha M. Deshpande
Department of Periodontology and Implantology, CSMSS Dental College and Hospital, Aurangabad, Maharashtra 431136,India.
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