Atypical chronic rhinosinusitis in Papillon syndrome -Lefevre: Presentation of 2 cases

Abstract

Papillon-Lefebre syndrome (SPL) is an extremely rare autosomal recessive disorder characterized by palmoplantar keratoderma and periodontitis, with an estimated incidence of 1-4 cases per million. Patients with SPL are highly susceptible to infection. The etiology of this susceptibility is unknown; however, an association with defects in neurophilic dysfunction, insufficient response of lymphocytes, defects in monocyte functions and deterioration of cytotoxic function of NK cells has been suggested. The manifestations include oral cavity specifically at the dental level, however at the level of paranasal sinuses there is a singular manifestation little known, as our two cases. We propose the criteria of Lugo cols. for diagnosis