Abstract
The study aims to present the diagnosis and treatment results of a case of Papillon–Lefevre syndrome. A clinical observation of Papillon–Lefevre syndrome, one of the genetic cornification disorders, is described. Clinical and anamnestic data, the results of examination and therapy are presented. The patient diagnosed with genetic keratoderma, which was affecting her life quality due to the severity of clinical manifestations and dental disorders, was being observed. The patient was unable to receive comprehensive therapy for an extended period of time due to her pregnancy and lactation. After her reproductive period was over, the patient was given a course of systemic acitretin therapy with a significant positive effect. The study proves the importance of early diagnosis and pathogenetic treatment.
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