Syndromes That Include Both Palmoplantar Keratoderma And Severe Periodontitis: A Review

R.H Dababneh

doi:10.4172/2161-1122.1000186

Abstract

Palmoplantar keratodermas (PPK) include a heterogeneous group of disorders with overlapping clinical features. The main aspect of PPK is thickening and hyperkeratosis of the palmar and plantar skin, that may be hereditary or acquired; diffuse, focal, or punctuate; and transgrediens or progrediens. PPKs are further distinguished by their mode of inheritance and by the presence of certain associated clinical features. Periodontitis was reported in association with more than one syndrome characterized by PPK. An extensively reported one is the Papillon-Lefevre syndrome (PLS) which is characterized by early onset of PPK and periodontitis affecting the primary and secondary dentitions. In addition to PLS, Haim-Munk, HOPP, Variant Carvajal and Weary-Kindler are other syndromes manifested by PPK and reported in association with severe periodontitis. Atypical cases of PLS were also reported, such as partial expression or a late presentation of the syndrome. The aim of this articleis to critically review the literature concerned with Papillon-Lefevre syndrome in its typical and atypical clinical presentation, in addition to other syndromes manifested at the same time by PPK and severe periodontitis. Thorough history and medical examination, together with periodontal, dermatologic, and genetic counseling, are important to exclude other existing medical conditions or other syndromes that might need special attention and care.

Highlights

Periodontal disease is a spectrum of different diseases for which certain individuals are at relatively high risk [1]
Kindler syndrome is an autosomal recessive disorder characterized by epidermolysis bullosa, congenital poikiloderma, Palmoplantar Keratoderma (PPK), photosensitivity, skin atrophy, and mucosal lesions [75] whereas Weary-Kindler syndrome is a dominantly inherited disorder with features similar to those reported in the Kindler syndrome except for skin atrophy, photosensitivity, and mucosal lesions [76]
Genetic syndromes may be manifested by PPK or by periodontitis, but Papillon-Lefévre syndrome is the most recognized syndrome sharing these two features

Summary

Introduction

Periodontal disease is a spectrum of different diseases for which certain individuals are at relatively high risk [1]. Kothiwale and Mathur in 2008 reported a different case of partial expression of PLS for a 35 year-old male with early onset PPK, but the eruption and exfoliation of the deciduous dentition was normal along with normal eruption of the permanent teeth. Kindler syndrome is an autosomal recessive disorder characterized by epidermolysis bullosa, congenital poikiloderma, PPK, photosensitivity, skin atrophy, and mucosal lesions [75] whereas Weary-Kindler syndrome is a dominantly inherited disorder with features similar to those reported in the Kindler syndrome except for skin atrophy, photosensitivity, and mucosal lesions [76] This syndrome was reported in a 13-years old girl who had been under long term dental care for prepubertal periodontitis, premature root resorption of primary teeth, soft tissue and dental anomalies, and angular cheilitis [77]

Conclusion

Typical types of PLS include