Atypical familial Papillon-Lefevre syndrome
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Tarih
2001
Yazarlar
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
European Acad Dermatology & Venereology
Erişim Hakkı
info:eu-repo/semantics/closedAccess
Özet
The Papillon-Lefevre syndrome is a rare autosomal recessive disorder. Consanguinity seems a notable prerequisite. Papillon-Lefevre 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-Lefevre 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.
Açıklama
Anahtar Kelimeler
Hyperkeratosis, Papillon-Lefevre Syndrome, Periodontitis, Psoriasiform Lesions, Retinoids
Kaynak
Journal of The European Academy of Dermatology and Venereology
WoS Q Değeri
Q3
Scopus Q Değeri
Q1
Cilt
15
Sayı
1
