English

Abstract

Papillon-Lefevre syndrome (PLS) is a very rare syndrome of autosomal recessive inheritance characterized by progressive palmar-plantar keratoderma and early onset periodontitis, leading to premature loss of both primary and permanent dentitions and recurrent pyogenic infections. Patient Presented with a chief complaint of thickening of palms and soles extending on to knees and elbows.. On intraoral examination there is loss of both temporary and permanent teeth. Patient had recurrent pyogenic infections involving face and nose leading to nasal destruction. Histopathological examination of the specimen taken from the thickened skin was reported to be consistent with PLS1,2,3