CASE REPORT OF IMPORTANT ORO-FACIAL CONSEQUENCES RESULTING FROM A LATE DIAGNOSIS OF PAPILLON-LEFEVRE SYNDROME

Papillon-Lefevre syndrome is a rare genodermatosis characterized by palmoplantar keratoderma and severe destructive periodontal disease. Clinically, it is characterized by hyperkeratosis on soles of the feet and sometimes on palms of hands and includes a transgradient pattern with scattered psoriasiform lesions. Following the exfoliation of primary teeth, periodontal destruction severely affects the alveolar bone in which the permanent dentition is retained, resulting in severe atrophy of the alveolar ridges. As a result, patients developed edentulism early in adolescent years. Other symptoms may include hyperhidrosis, intracranial calcification, arachnodactyly, susceptibility to infections and mental retardation. We here in report a rare case presentation of a 15 year old male Ecuadorian patient who suffers from sharply demarcated erythematous and scaly plaques in palms, forearms, knees and low back since childhood. Furthermore, the patient was partially edentulous as evidence of aggressive periodontitis was present.