From Big Medical Encyclopedia

LIEFIEVRA-PAPIYONA SYNDROME (P. Lefevre, fr. dermatologist, sort. in 1891; M of M. of Papillon, sovr, the fr. dermatologist) — the symptom complex which is characterized by the formation of pathological dentogingival pockets, shaking and loss of teeth expressed by keratinization of palms and soles. It is described for the first time in 1924 by Papiyonom and Lefebvre.

Consider that the symptom complex is a consequence of deep neuroendocrinal disturbances. Display of this disease at several members of one family is noted, however it was not succeeded to prove the hereditary nature it.

The disease represents a combination of the symptoms characteristic of a severe form of periodontosis and a keratodermia (see. Keratoza ). Development of a disease is possible at children's age (3 — 6 years), at the age of 14 — 15 years and later. At children the disease begins with shaking and loss (in 2 — 3) milk teeth, and then and second teeth — soon after their eruption owing to destruction of a bone tissue in the field of alveoluses of jaws, and also an ulitis — education patol, dentogingival pockets with the purulent separated, .znachitelny hypostasis and bleeding of gums. Teeth are mobile, displaced. In some cases at adults on a mucous membrane of an oral cavity sites of keratinization (leukoplakia) can be observed.

Radiological the atrophy of sites of an alveolar shoot of an upper jaw and alveolar edge of a mandible is defined. Defeat has the progressing character and usually leads in several years to full loss of teeth.

Along with changes in an oral cavity the keratodermia of palms and soles develops: skin becomes thick, dry with the expressed peeling and fracturing. Skin of extensor surfaces of elbow and knee joints is exposed to noticeable keratinization (see Keratoza). Change of nails is noted — they become uneven, dim. Increase limf, nodes, dysfunction of a thyroid gland is possible.

Diagnosis put on the basis of typical a wedge, pictures. The differential diagnosis is carried out with eosinophilic granuloma (see), periodontosis (see).

Treatment symptomatic. Make removal of tooth deposits, a scraping patol, dentogingival pockets, if necessary excision patholologically of the changed tissues of a gum and an exodontia with mobility of the III degree. At the same time appoint a course of treatment vitamins: Aevitum, A, B 1 , In 6 , In 12 , With, retinonovy to - that; outside treatment of sites of a keratosis; according to indications — drugs of a thyroid gland. Carry out denture treatment (removable prostheses), careful care of an oral cavity is obligatory.

The forecast for life favorable.

Bibliography: Zhits M. 3., Grebennikov V. A. and Litakova G. I. Sluchay of a keratodermia like Papiyon — Lefebvre, Klin, medical, t. 55, No. I, page 129, 1977; Kunin A. A. A case of a syndrome of Papil-on — Lefebvre, Stomatology, t. 52, No. 3, page 74, 1973; Papillon et Lefevre P. Deux cas de keratodermie palmaire et plantaire symetrique familiale (maladie de Meleda) chez le fr^re et la soeur, coexistence dans les deux cas d’alterations den-taires graves, Bull. Soc. frang. Derm. Syph., t. 31, p. 82, 1924.

L. P. Ruby.