Table of Content


Spring 2019, Vol. 27 No. 1

Hong Kong J. Dermatol. Venereol. (2019) 27, 31-35

Case Report

Papillon-Lefevre syndrome: a rare case report and review of literature


D Vupperla, P Elaprolu, FT Taj


Papillon-Lefevre syndrome (PLS) is a rare genetically inherited autosomal recessive disorder. A 34-year-old woman presented with complaints of diffuse hyperkeratosis with scaling over both palms and soles with sclerodactyly of all fingers, increased sweating, loss of teeth with periodontitis and recurrent skin infections. History of first-degree consanguinity among the patients was present. Loss of teeth can be prevented by prompt administration of oral retinoids during the eruption of permanent teeth. Despite meticulous attention, many patients are still identified with PLS. Therefore, clinicians should be aware of this entity to avoid delay in diagnosis.


Keywords: Cathepsin C, hyperkeratosis, Palmoplantar keratoderma, Periodontitis, Pyoderma

關鍵詞: 組織蛋白酶C、角化過度、掌蹠角化病、牙周炎、膿皮病