Gardner — Diamond Sendromu (Oto - Eritrosit Duyarlık Sendromu)

Year 1974, Volume: 27 Issue: 1-2, 344 - 351, 30.06.1974

Ayhan Çavdar

Abstract

Papillon - Lefevre sendromu gösteren 6 yasında bir erkek çocuk sunulmustur. Palmar - plantar keratoz, dislterin erken periodontal destrüksiyonuna ek olarak gelisme geriliği, hipertelorizm, burun kökü basıklığı, iç epikantus, kemik yasında gerilik, çocukluğunda geçirilmis piyodermi ve sık bronkopnömoni hikâyesi vardl. Ektopik kalsifikasyon görülmedi. Anne, baba araslnda 1. dereceden akraballk bulunmasl otosomal resessif geçisi desteklemektedir

Keywords

otozomal resesif , papillon , levefre

Gardner — Dıamond Syndrome (Auto - Erythrocyte Sensitivity Syndrome)

Abstract

A case with Papillon - Lefevre Syndrome has been presented. The patient showed palmo - plantar keratosis with premature periodontal destruction of the teeth. In addition to these, he also had failure to thrive, hypertelorism, depressed nasal bridge, inner epicanthal folds, retarded bone age, and a history of recurrent pyodermia and. bronchopneunomia. There was not any ectopic calfification. Parental consanquinity implies the autosomal recessive mode of inheritance.

Keywords

autosomal recessive , papillan , lefevre

References

• 1 — Papillon, M. M., Lefevre, P. : Deux cas de keratodermie palrnaire et palantaire symetrique familiale (Maladie de Meleda( Chez le frére et la soeur. Coexistence dans les deux cas d'altérations dentaires graves. Soc. Franc. Dermat. et Syph. 31:82, 1924.
• 2 — Gorlin, R. J. , Sedano, H. and Anderson, V. E. : Syndrome of palmarplantar hyperkeratosis and premature periodontal destruction of the teeth. J. Pediat. 65:895, 1965.
• 3 Gedda, L. and Pignatelli, A. : Cheratosi Palmo - Plantare e Allotopa Associata a Grave Mallattia Dentaria in due fratelli Monanati. Acta Genet. Med. e Gemell, 3:133, 1954.
• 4 Bejcek, Z, : Hyperkeratosis palmo - plantaris cum paradentosis seu morbus Papillon Lefevre. C. Stomat. 60: 198, 1960.
• 5 — Boisrame, M. R. : Polyalveolyse chez deux enfants atteintes d'une keratodermie palmoplantaire. Rev. Stomat. 54:242, 1953.
• 6 — Corson, F. : Keratosis palmaris et plantaris with dental alterations. Dermat. and Syph. 40:639, 1939.
• 7- Jansen, L. H. and Dekker, G. : Hyperkeratosis palmo - plantaris with periodontosis (Papillon - Lefevre) Dermatologica 113 :207, 1956.
• 8 mprkowski, L., Yochanan, R. and Brish, M. : Hyperkeratosis palmoplantaris with periotontosis (Papillon - Lefevre). Arch. Dermat. 88:207, 1963.
• 9 — Dekker, G. and Jansen, L. H. : Periodontosis in a child with hyperkeratosis palmo - plantaris. J. Perio. 29:266, 1958.
• 10 — Coccia, C. T. , McDonald, R. E. , Mitchell, D. G. : Papillon - Lefevre syndrome : Précocious periodontosis with palmar hyperkeratosis. J. Periodont 37•.408, 1966.
• 11 — Wannenmacher, E. : Umschau auf dem Gebiet der Paradentose. Zentralbl. für gesamt. Zahn - Mund und Kieferheilkunde 3:81, 1938.
• 12 — Wannenmacher, E. : Umschau auf dem Gebiet der Paradentopathien. Zentralbl, gesamt. Zahn, Mund - und Kieferhelikunde 7:1, 1942.
• 13 — Bingöl, 0., Güney, O. : Papillon - Lefevre Sendromu. Çocuk sagllgl ve Hastahklarl Dergisi, 11:131, 1968.
• 14 Rainova, I. : Über insufficientia Parodontalis (Parotontosis, Amphodon„ tosis bei Kindern). Deutcshe Stomat, 5:681, 1955.
• 15 — Wyan, H. : Die dystrophische Parodontosis im Milchgebiss als Manifestation einer ektodermalen Acropathie (Parotontopathia acroectodermalis) Deutsche Stomat. 7:342, 1957.
• 16 — Lenstrup, n. and Brinih, O. : Ein ungewöhnlioher Fall von jugendlicher Paradentose. Paradentium 9:102, 1037.
• 17 — Hawes, R. P. : Report of three patients experiencing juvenile periodontosis and early loss of teeth. J. Dent. Child. 27:169, 1969.
• 18 — Naegeli, O. : Familiarer chromatophorennaevus. Schweiz. Med. Wchnschr. 57:48, 1927.
• 19 — Krepler, P. : Pachyonychia congenita jadossohn - lewandavsky Helvet. Paediat. Acta 10•.369, 1955.
• 20 — Spannlang, H. : Beitrage zur klinik und pathologie seltener Hornhauterkrankungen. Ztschr. Augenheilk, 62•.21, 1927.
• 21 — Wohwinkel, K. H. : Keratoma hereditarium mutilans. Arch. Dermat. u. syph (Berlin) 158:354, 1929.
• 22 — Brunauer; S. R. : Zur Symptomatologie und Histologie der kongenitalen Dykeratosen. Dermat. Ztschr. 42:6, 1925.
• 23 — Volavsek, W. : Zur Klinmç der Nagelveranderungen und Palmarkeratosen bei Syringomyelia. Arch. Dermat. Syph (Berlin) 182 :52, 1941.
• 24 — Silverman, S., Renstrup, G. , Pindborg, J. J. : Studeies in oral leukoplakias. Ill. Affecta of vitamin A comparing clinical, histopathologic, cytologic and hematologic responses. Acta Odon. Scandinav, 21:271, 1963.