Related Experiment Video
Updated: Aug 11, 2026

Analyses of Proteinuria, Renal Infiltration of Leukocytes, and Renal Deposition of Proteins in Lupus-prone MRL/lpr Mice
Published on: June 8, 2022
A case of Papillon-Lefevre syndrome associated with xanthogranulomatous pyelonephritis and hepatitis
A Tülin Mansur1, Fatih Göktay, Nüket Demirok
1Department of Dermatology, Haydarpaşa Numune Training and Research Hospital, Istanbul, Turkey. tulinmansur@hotmail.com
Abstract:
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.
Related Concept Videos
Acute Pyelonephritis I: Introduction
Acute Pyelonephritis II: Diagnostic Studies and Management
Appendicitis
Gastritis II: Pathophysiology
Hepatitis
Chronic Pancreatitis I: Introduction