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Anais Brasileiros de Dermatologia
On-line version ISSN 1806-4841
COELI, Flávia Regina et al. Do you know this Syndrome?. An. Bras. Dermatol. [online]. 2008, vol.83, n.4, pp. 375-377. ISSN 1806-4841. http://dx.doi.org/10.1590/S0365-05962008000400015.
Papillon-Lefèvre syndrome, or palmoplantar keratoderma with periodontitis, is a rare genodermatosis with cutaneous and dental repercussions. The condition appears at around the first year of life, with transgressive palmar-plantar keratoderma associated with periodontitis, early loss of both deciduous and permanent teeth. It is frequently associated with pyogenic infection of the skin and internal organs. Histopathology is nonspecific, the diagnosis being eminently clinical.
Keywords : Dipeptidyl peptidase I; Periodontitis; Retinoids.