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Papillon-Lefèvre syndrome: clinical presentation and management options.

Sreeramulu B, Shyam ND, Ajay P, Suman P - Clin Cosmet Investig Dent (2015)

Bottom Line: Papillon-Lefèvre syndrome (PLS) is a rare autosomal recessive disorder, characterized by diffuse palmoplantar keratoderma and precocious aggressive periodontitis, leading to premature loss of deciduous and permanent dentition at a very young age.Various etiopathogenic factors are associated with the syndrome, like immunologic alterations, genetic mutations, and the role of bacteria.Dentists play a significant role in the diagnosis and management of PLS as there are characteristic manifestations like periodontal destruction at an early age and an early eruption of permanent teeth.

View Article: PubMed Central - PubMed

Affiliation: Department of Prosthodontics, Government Dental College and Hospital, Hyderabad, Telangana State, India.

ABSTRACT
Papillon-Lefèvre syndrome (PLS) is a rare autosomal recessive disorder, characterized by diffuse palmoplantar keratoderma and precocious aggressive periodontitis, leading to premature loss of deciduous and permanent dentition at a very young age. Various etiopathogenic factors are associated with the syndrome, like immunologic alterations, genetic mutations, and the role of bacteria. Dentists play a significant role in the diagnosis and management of PLS as there are characteristic manifestations like periodontal destruction at an early age and an early eruption of permanent teeth. Here, we are presenting an elaborate review of PLS, its etiopathogenesis, clinical presentation, and management options.

No MeSH data available.


Related in: MedlinePlus

Hyperkeratosis of dorsal surfaces of the hands and feet.
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f2-ccide-7-075: Hyperkeratosis of dorsal surfaces of the hands and feet.

Mentions: Cutaneous lesions or skin lesions of PLS (keratoderma) are usually manifested simultaneously with oral manifestations between the ages of 6 months and 4 years, coinciding with eruption of deciduous teeth. At the beginning, thickening of the skin is present; later, skin lesions are well demarcated extending to thenar and hypothenar eminences of palms, to the Achilles tendon and external malleoli of feet (Figure 2). The dorsal surface of fingers and toes as well as the elbows, knees, legs, and thighs are less severely affected, and the trunk is rarely affected. Lesions may vary in colour, texture, and manifestation; they appear as white, brown, red, and scaly patches which undergo crustation, cracking, and deep fissuring (Figure 3). Skin lesions get aggravated during cold weather and patients experience pain when walking. Infection may superimpose the defective skin leading to formation of abscesses. Other findings are retardation of somatic development, follicular keratosis and hyperhidrosis (bromhidrosis), nail dystrophy, and calcification of falx cerebri and choroid plexus.40


Papillon-Lefèvre syndrome: clinical presentation and management options.

Sreeramulu B, Shyam ND, Ajay P, Suman P - Clin Cosmet Investig Dent (2015)

Hyperkeratosis of dorsal surfaces of the hands and feet.
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4507741&req=5

f2-ccide-7-075: Hyperkeratosis of dorsal surfaces of the hands and feet.
Mentions: Cutaneous lesions or skin lesions of PLS (keratoderma) are usually manifested simultaneously with oral manifestations between the ages of 6 months and 4 years, coinciding with eruption of deciduous teeth. At the beginning, thickening of the skin is present; later, skin lesions are well demarcated extending to thenar and hypothenar eminences of palms, to the Achilles tendon and external malleoli of feet (Figure 2). The dorsal surface of fingers and toes as well as the elbows, knees, legs, and thighs are less severely affected, and the trunk is rarely affected. Lesions may vary in colour, texture, and manifestation; they appear as white, brown, red, and scaly patches which undergo crustation, cracking, and deep fissuring (Figure 3). Skin lesions get aggravated during cold weather and patients experience pain when walking. Infection may superimpose the defective skin leading to formation of abscesses. Other findings are retardation of somatic development, follicular keratosis and hyperhidrosis (bromhidrosis), nail dystrophy, and calcification of falx cerebri and choroid plexus.40

Bottom Line: Papillon-Lefèvre syndrome (PLS) is a rare autosomal recessive disorder, characterized by diffuse palmoplantar keratoderma and precocious aggressive periodontitis, leading to premature loss of deciduous and permanent dentition at a very young age.Various etiopathogenic factors are associated with the syndrome, like immunologic alterations, genetic mutations, and the role of bacteria.Dentists play a significant role in the diagnosis and management of PLS as there are characteristic manifestations like periodontal destruction at an early age and an early eruption of permanent teeth.

View Article: PubMed Central - PubMed

Affiliation: Department of Prosthodontics, Government Dental College and Hospital, Hyderabad, Telangana State, India.

ABSTRACT
Papillon-Lefèvre syndrome (PLS) is a rare autosomal recessive disorder, characterized by diffuse palmoplantar keratoderma and precocious aggressive periodontitis, leading to premature loss of deciduous and permanent dentition at a very young age. Various etiopathogenic factors are associated with the syndrome, like immunologic alterations, genetic mutations, and the role of bacteria. Dentists play a significant role in the diagnosis and management of PLS as there are characteristic manifestations like periodontal destruction at an early age and an early eruption of permanent teeth. Here, we are presenting an elaborate review of PLS, its etiopathogenesis, clinical presentation, and management options.

No MeSH data available.


Related in: MedlinePlus