Research Article| Volume 24, ISSUE 2, P180-185, August 1967

Hyperkeratosis palmoplantaris with periodontosis (Papillon-Lefèvre syndrome)

Report of a case
      This paper is only available as a PDF. To read, Please Download here.


      Hyperkeratosis palmoplantaris with periodontosis (Papillon-Lefèvre syndrome) in a 10-year-old Negro boy is presented. Fewer than fifty cases have been reported in the world's literature. The classic findings of hyperkeratotic skin lesions on the palms and soles, associated with destruction of the periodontium of the deciduous and permanent teeth leading to dental exfoliation, are shown. The acute gingival inflammation accompanying the latter aspect of the syndrome is discussed. The etiologic role of parental consanguinity and genetics is mentioned, although not proved in the case presented here. The disease is progressive, resulting in loss of the permanent dentition (with the exception of the third molars) and mild to severe lesions of the skin. Treatment is symptomatic and not always satisfactory.
      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


        • Papillon H.M.
        • Lefèvre P.
        Deux cas de keratodermie palmaire et plantaire symetrique familiale (maladie de meleda) chez de frère et la loer; coexistance dans le deux cas d'alterations dentaíres graves.
        Bull. Soc. franç. dermat. et syph. 1924; 31: 82-87
        • Gorlin R.J.
        • Sedano H.
        • Anderson V.E.
        The Syndrome of Palmar-Plantar Hyperkeratosis and Premature Periodontal Destruction of the Teeth.
        J. Pediat. 1964; 65: 895-908
        • Greither A.
        Keratosis Palmo-plantaris mit Periodontopathie (Papillon-Lefèvre).
        Dermatologica. 1959; 119: 248-263
        • Jansen L.H.
        • Dekker G.
        Hyperkeratosis Palmo-plantaris with Periodontosis (Papillon-Lefèvre).
        Dermatologica. 1956; 113: 207-219
        • Jansen L.H.
        Le caractere heréditaire de l'hyperkeratose palmo-plantaire avec paradentose (Papillon-Lefèvre).
        J. Genet. Hum. 1956; 5: 216-224
        • Dekker G.
        • Jansen L.H.
        Periodontosis in a Child With Hyperkeratosis Palmoplantaris.
        J. Periodont. 1958; 29: 266-271
        • Brunsting L.A.
        Palmer Plantar Hyperkeratosis With Periodontosis (Papillon-Lefèvre Syndrome).
        Arch. Dermat. 1964; 90 (and others): 330
        • Ziprowski L.
        • Yochanan R.
        • Brish M.
        Hyperkeratosis Palmo-plantaris With Periodontosis (Papillon-Lefèvre).
        Arch. Dermat. 1963; 88: 207-209
        • Haim S.
        • Munk J.
        Keratosis Palmo-plantaris Congenita, With Periodontosis, Arachnodactyly and a Peculiar Deformity of the Terminal Phalanges.
        Brit. J. Dermat. 1965; 77: 42-54