Lipoid proteinosisli iki olguda tekrarlayan oral ülserler

Year 2012, Volume: 9 Issue: 2, 71 - 72, 01.08.2012

Selma Bakar Dertlioglu Demet Cicek İlyas Ozardalı

Abstract

Lipoid proteinozis LP nadir görülen otosomal resesif bir genodermatoz olup deri, oral kavite, larinks ve iç organlarda amorf hyalin kristal materyal birikimi ile karakterizedir. Birikim özellikle deride olmak üzere, üst solunum-sindirim sistemi mukozası ve iç organlarda olur. Hastalarımıza klinik bulgular, aile öyküsü ve histopatolojik değerlendirme sonucu LP tanısı konuldu. Hastaların dilinde tekrarlayan ağrılı ülserler mevcut olup nadir rastlanılan bu vakaları LP oral ülser birlikteliği açısından sunmayı uygun bulduk

Keywords

Lipoid proteinozis, oral ülser, dil

Two cases of recurrent oral ulcers with lipoid proteinosis

Abstract

Lipoid proteinosis LP is a rare autosomal recessive disorder characterized by infiltration of amorphous hyaline material in the skin, oral cavity, larynx, and internal organs. Deposition may be in different parts of the body, especially in the skin, mucous membranes of the upper aero digestive tract, and internal organs. LP was diagnosed with clinical findings, family history and histopathological evaluation. LP may be very rarely associated with oral ulcers. In this report, we aim to describe two cases of patients affected by LP associated with painful and recurrent ulcerative lesions in the tongue

Keywords

Lipoid proteinosis, oral ulcers, tongue

References

• Touart DM, Sau P. Cutaneous deposition diseases. Part I. J Am Acad Dermatol 1998; 39: –71
• Chan I, Liu L, Hamada T, Sethuraman G, McGrath JA. The molecular basis of lipoid proteinosis: mutations in extracellular matrix protein 1.Exp Dermatol 2007; 16: 881–90
• Aroni K, Lazaris AC, Papadimitriou K, Paraskevakou H, Davaris PS. Lipoid proteinosis of the oral mucosa: case report and review of the literature. Pathol Res Pract 1998; 194: 855–9
• Disdier P, Harlé JR, Andrac L, Swiader L,Weiller PJ. Specific xerostomia during Urbach–Wiethe disease. Dermatology 1994; 188: 50–1
• Bazopoulou-Kyrkanidou E, Tosios KI, Zabelis G, Charalampopoulou S, Papanicolaou SI. Hyalinosis cutis et mucosae: gingival involvement. J Oral Pathol Med 1998; 27: 233-7
• Sargenti Neto S, Batista JD, Durighetto AF Jr. A case of oral recurrent ulcerative lesions in a patient with lipoid proteinosis (Urbach-Wiethe disease). Br J Oral Maxillofac Surg 2010; 48: 654-5
• Kaya TI, Gunduz O, Kokturk A, Tursen U, Ikizoglu G. A life-threatening exacerbation of lipoid proteinosis. J Eur Acad Dermatol Venereol 2002; 16: 286–8
• Ramsey ML, Tschen JA, Wolf JE Jr. Lipoid proteinosis. Int J Dermatol 1985; 24: 230-2.
• Harran Üniversitesi Tıp Fakültesi Dergisi (Journal of Harran University Medical Faculty) Cilt 9. Sayı 2, 2012