Progressive systemic sclerosis is an autoimmune disease characterized by gradual deposition of abnormal collagen which causes fibrous changes of the skin and visceral organs such as lung, heart, and kidney, which lead to serious complication. Microangiopathy and firm skin cause extrinsic pressure and obliteration of the vessels that lead to ischemia and destruction of the underlying bone. Salivary gland fibrosis causes xerostomia that increases risk of dental caries. Therefore, oral hygiene instruction (OHI) and preventive dentistry are recommended for these patients. We present a 36-year-old female that suffers from systemic sclerosis with total lysis of the mandibular condyle. 1. Introduction Scleroderma or progressive systemic sclerosis (PSS) was first reported in 1752 by Curzio of Naples [1]. PSS, also known as scleroderma, is a connective tissue disorder characterized by fibrosis of the skin and visceral organs [2]. The cause of PSS is immunologically mediated pathogenesis. PSS occurs predominately in the adult but it can also be seen in childhood, with females affected three to four times as often as males [3]. The most common manifestations is microstomia that causes a limitation of opening the mouth in two-thirds of the cases. In addition, salivary gland fibrosis causes xerostomia; therefore, these patients are more prone to dental decay. In some patients, as loss of attachment gingival occurs, multiple area of gingival recession may be present. In scleroderma patients, preventive care is of foremost importance. Meticulous oral hygiene must be established and maintained. Occasionally slight bilateral erosion of mandibular condyle causes anterior open bite. Bone resorption is caused by the increased pressure associated with the deposition of collagen. Bone resorption has been reported in maxillofacial region, which occurs in masticatory muscle attachments [4]. On radiographic appearance a characteristic resorption of the ramus and condyle may be observed. We reported a case of total osteolysis of the mandibular condyle in a 36-year-old female patient with oral manifestations of the disease. 2. Case Report A 36-year-old female suffering from PSS with 9-year history of disease was referred to oral and maxillofacial surgery for extraction of her decayed posterior teeth. The patient had reduced maximal interincisal opening (MIO = 12？mm), mask-like face, perioral deep rhytides, pinched nose, facial telangiectasia, and atrophic ulcerated finger tips (sclerodactyly). Pain accompanying bone resorption is present in her fingers (painful digital ischemia).
References
[1]
W. P. Naylor, “Oral management of the scleroderma patient,” The Journal of the American Dental Association, vol. 105, no. 5, pp. 814–817, 1982.
[2]
M. M. Rubin and R. J. Sanfilippo, “Resorption of the mandibular angle in progressive systemic sclerosis: case report,” Journal of Oral and Maxillofacial Surgery, vol. 50, no. 1, pp. 75–77, 1992.
[3]
M. A. Z. de Figueiredo, J. A. P. de Figueiredo, and S. Porter, “Root resorption associated with mandibular bone erosion in a patient with scleroderma,” Journal of Endodontics, vol. 34, no. 1, pp. 102–103, 2008.
[4]
H. ？ankaya and Y. Kabasakal, “Mandibular resorption due to progressive systemic sclerosis: a case report,” Journal of Oral and Maxillofacial Surgery, vol. 59, no. 5, pp. 565–567, 2001.
[5]
C. Cazal, A. P. V. Sobral, R. F. S. N. Neves, F. W. V. Freire Filho, á. B. Cardoso, and M. M. F. da Silveira, “Oral complaints in progressive systemic sclerosis: two cases report,” Medicina Oral, Patologia Oral y Cirugia Bucal, vol. 13, no. 2, pp. 114–118, 2008.
[6]
S. C. White, N. W. Frey, D. D. Blaschke et al., “Oral radiographic changes in patients with progressive systemic sclerosis (scleroderma),” The Journal of the American Dental Association, vol. 94, no. 6, pp. 1178–1182, 1977.
[7]
W. P. Naylor, “Oral management of the scleroderma patient,” The Journal of the American Dental Association, vol. 105, no. 5, pp. 814–817, 1982.
[8]
R. E. Wood and P. Lee, “Analysis of the oral manifestations of systemic sclerosis (scleroderma),” Oral Surgery Oral Medicine and Oral Pathology, vol. 65, no. 2, pp. 172–178, 1988.
[9]
M. M. Rubin and R. J. Sanfilippo, “Resorption of the mandibular angle in progressive systemic sclerosis: case report,” Journal of Oral and Maxillofacial Surgery, vol. 50, no. 1, pp. 75–77, 1992.
[10]
F. E. Hopper and A. D. Giles, “Orofacial changes in systemic sclerosis. Report of a case of resorption of mandibular angles and zygomatic arches,” British Journal of Oral Surgery, vol. 20, no. 2, pp. 129–134, 1982.
[11]
R. G. Jagger, E. G. Absi, H. G. Jeremiah, and A. W. Sugar, “Bilateral mandibular condylysis in a patient with systemic sclerosis,” Dentomaxillofacial Radiology, vol. 35, no. 6, pp. 461–463, 2006.
[12]
P. E. Haers and H. F. Sailer, “Mandibular resorption due to systemic sclerosis. Case report of surgical correction of a secondary open bite deformity,” International Journal of Oral and Maxillofacial Surgery, vol. 24, no. 4, pp. 261–267, 1995.
[13]
H. I. Caplan and R. A. Benny, “Total osteolysis of the mandibular condyle in progressive systemic sclerosis,” Oral Surgery Oral Medicine and Oral Pathology, vol. 46, no. 3, pp. 362–366, 1978.
[14]
E. Alp？z, H. Cankara, and P. Güneri, “Facial subcutaneous calcinosis and mandibular resorbtion in systemic sclerosis: a case report,” Dentomaxillofacial Radiology, vol. 36, pp. 172–174, 2007.
[15]
G. Nagy, J. Kovács, M. Zeher, and L. Czirják, “Analysis of the oral manifestations of systemic sclerosis,” Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology, vol. 77, pp. 141–146, 1994.
