Orofacial findings in chronic granulomatous disease: report of twelve patients and review of the literature

Abstract

BackgroundChronic granulomatous disease is an extremely rare primary immunodeficiency syndrome that can be associated with various oral complications. This can affect high number of patients. However, data on oral complications is sparse. Here we will review the literature and describe the orofacial findings in 12 patients.FindingsThe age range was 5-31 years. Oral findings were variable, and reflected a low level of oral hygiene. They included periodontitis, rampant caries, gingivitis, aphthous-like ulcers, and geographic tongue. One patient had white patches on the buccal mucosa similar to lichen planus. Another patient had a nodular dorsum of the tongue associated with fissured and geographic tongue. Biopsies from the latter two lesions revealed chronic non-specific mucositis. Panoramic radiographs showed extensive periodontitis in one patient and periapical lesions in another patient.ConclusionPatients with chronic granulomatous disease may develop oral lesions reflecting susceptibility to infections and inflammation. It is also possible that social and genetic factors may influence the development of this complication. Therefore, oral hygiene must be kept at an optimum level to prevent infections that can be difficult to manage.

Gingivitis and Very High IgE Level in a Chronic Granulomatous Disease Patient with Unusual Presentation: A Case Report

“…2,[16][17][18][19] However, Dar-Odeh et al reported presence of oral complications in 58% of their 12 CGD patients and biopsies of 2 showed chronic nonspecific mucositis. 20 Our patient presenting with the AR form of CGD had troublesome periodontal complaints and came to medical attention by the presence of chronic gingivitis, although he had a crowded history of serious infections. His serum IgE levels were high and he was referred to our unit with the probable diagnosis of HIES.…”

Normal Variations of Oral Anatomy and Common Oral Soft Tissue Lesions

“…Fissured tongue has been seen more in individuals with Down syndrome than the general population. 57 Fissured tongue is a component of Melkerson-Rosenthal syndrome, characterized by the classic triad of facial palsy, facial swelling, and fissured tongue. 56,58-60 Fissured tongue is a benign condition and no specific treatment is indicated.…”

Manifestaciones clínicas de las hemopatías no neoplásicas en Odontología

“…La enfermedad granulomatosa crónica cursa con candidiasis, ulceraciones con pseudomembranas en cualquier parte de la mucosa oral, sinusitis recurrentes, linfadenitis cervicales, queilitis eccematosa, gingivitis intensa, lengua geográ fica, hipoplasia del esmalte, caries rampantes e infecciones cutá neas faciales 14,15 . Hay autores que la relacionan tambié n con la aparició n de periodontitis prepuberal generalizada 16 ; la periodontitis prepuberal se ha relacionado con una deficiencia en la adhesió n de los leucocitos 15 .…”

“…Hay autores que la relacionan tambié n con la aparició n de periodontitis prepuberal generalizada 16 ; la periodontitis prepuberal se ha relacionado con una deficiencia en la adhesió n de los leucocitos 15 . Muchas de las manifestaciones se relacionan con factores gené ticos o sociales, como la falta de higiene oral, por lo que se recomienda un cuidado dental especializado y regular 14 .…”