Papillon–Lefèvre syndrome

[tirath]

Papillon–Lefèvre syndrome (PLS), also known as palmoplantar keratoderma with periodontitis, is an autosomal recessive.genetic disorder caused by a deficiency in cathepsin C.

PLS is characterized by periodontitis and palmoplantar keratoderma.The severe destruction of periodontium results in loss of most primary teeth by the age of 4 and most permanent teeth by age . Hyperkeratosis of palms and soles of feet appear in first few years of life

        

[sushantpatel_doc]

Any treatment besides symptomatic relief?

        

[tirath]

A multidisciplinary approach is important for the care of patients with PLS. The skin manifestations of PPK are usually treated with emollients. Oral retinoids such as acitretin, etretinate, andisotretinoin were reported to be beneficial for both dental and cutaneous lesions of PLS. Retinoid treatment may end up with normal dental development if started during eruption of permanent teeth.16 The periodontitis in PLS is usually difficult to control. Effective treatment for the periodontitis includes extraction of the primary teeth combined with oral antibiotics and professional teeth cleaning. It is reported that etretinate and acitretin modulate the course of periodontitis and preserve the teeth. A course of antibiotics should be tried to control the active periodontitis in an effort to preserve the teeth and to prevent bacteremia and subsequently pyogenic liver abscess

        

[Anonymous]

        

[tonyshori.perio]

it comes under the entity of periodontitis as a manifestation of systemic disease. it is best treated with conventional therapy along with antimicrobial therapy

        

[Author]

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