A case of Papillon-Lefevre syndrome associated with xanthogranulomatous pyelonephritis and hepatitis.
Papillon-Lefevre syndrome (PLS) is an autosomally recessive palmoplantar keratoderma accompanied by psoriasiform plaques on the extensor surfaces of extremities and leading to premature loss of deciduous and permanent teeth by progressive periodontitis. Patients with PLS may exhibit mental retardation, intracranial ectopic calcifications, nail dystrophies and a tendency to various infectious disorders, in addition to skin and oral findings. Herein, we report a 26-year-old man with PLS, who had experienced xanthogranulomatous pyelonephritis and hepatitis during childhood and adolescence. To the best of our knowledge, this is the first report of PLS associated with xanthogranulomatous inflammation.
Department of Dermatology, Haydarpaşa Numune Training and Research Hospital, Istanbul, Turkey. tulinmansur@hotmail.com
A Tülin
AT
Mansur
Fatih
F
Göktay
Nüket
N
Demirok
D000328:Adult; D003937:Diagnosis, Differential; D020022:Genetic Predisposition to Disease; D006505:Hepatitis / Q000150:complications / Q000175:diagnosis*; D006801:Humans; D008297:Male; D010214:Papillon-Lefevre Disease / Q000150:complications / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology; D011705:Pyelonephritis, Xanthogranulomatous / Q000150:complications / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology; D014463:Ultrasonography