Avtor/Urednik		Kansky, AA; Potočnik, M; Kansky, A
Naslov		Papillon-Lefevre syndrome (PLS)
Tip		članek
Vir		Acta Dermatovenerol Alp Pannon Adriat
Vol. in št.		Letnik 6, št. 2
Leto izdaje		1997
Obseg		str. 60-5
Jezik		eng
Abstrakt		Background. The syndrome was first described by Papillon and Lefevre in 1924, it is characterized by palmoplantar hyperkeratosis and severe periodontal breakdown, resulting in early loss of teeth. Objective. The authors were engaged in obtaining data on Papillon-Lefevre cases in the population of Slovenia. Methods. Clinical records were studied and patients' families were investigated at their homes. Among the 2 million Slovenian population 13 persons were detected in 7 families. Consanguinity could not be proven. 11 affected persons were monitored by the investigators. Results. The patients who cooperated in the study, showed in most cases psoriasis-like skin lesion and palmoplantar hyperkeratosis. A severe periodontitis appeared soon after the teeth eruption. Some of them were treated with antibiotics and retionids, nonetheless they lost all the teeth up to the end of puberty. Conclusion. The genetic defect remains unknown, The therapy is symptomatic: antibiotics and good dental cleaning are important. Retinoids influence primarily the skin lesions. Cooperation between dermatologist and stomatologist is needed and an early treatment is indicated.
Deskriptorji		PAPILLON-LEFEVRE DISEASE SLOVENIA ADOLESCENCE ADULT