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Atypical familial Papillon–Lefèvre syndrome
Author(s) -
Inalöz H Serhat,
Harman M,
Akdeniz S,
Inalöz SS,
Isik A Gulden
Publication year - 2001
Publication title -
journal of the european academy of dermatology and venereology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.655
H-Index - 107
eISSN - 1468-3083
pISSN - 0926-9959
DOI - 10.1046/j.1468-3083.2001.00121.x
Subject(s) - medicine , dermatology , hyperkeratosis , consanguinity , keratoderma , palmoplantar keratoderma , pathology , pediatrics
Abstract The Papillon–Lefèvre syndrome is a rare autosomal recessive disorder. Consanguinity seems a notable prerequisite. Papillon–Lefèvre syndrome manifests in the first 6 months of life with rapidly progressive periodontitis and severe alveolar bone destruction leading to early loss of both the deciduous and permanent teeth in association with palmo‐plantar hyperkeratosis. We present two unusual cases of familial Papillon–Lefèvre syndrome, one of whom has only late onset of mild skin lesions and the other has severe skin lesions and relatively mild periodontal disease. A number of other cases recently described have also had atypical features.