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CASE REPORT Table of Contents   
Year : 2007  |  Volume : 18  |  Issue : 1  |  Page : 27-30
Systemic sclerosis


Department of Periodontics, Meenakshi Ammal Dental College and Hospital, Alapakkam Main Road, Maduravoyal, Chennai - 600 095, India

Click here for correspondence address and email

Date of Submission20-Mar-2006
Date of Decision20-Sep-2006
Date of Acceptance05-Oct-2006
 

   Abstract 

Systemic sclerosis (scleroderma) is a rare generalized disorder of connective tissue origin. This condition is predominantly a clinical diagnosis, based on the clinical signs and symptoms. Here is a case report of 26-year-old female patient with the classical features of this disease. This case is reported for its rarity and variable expressivity. This article also reviews the literature of this uncommon condition.

Keywords: Acroosteolysis, collagen disease, crest syndrome, hidebound disease, scleroderma, systemic sclerosis

How to cite this article:
Ahathya R S, Deepalakshmi D, Emmadi P. Systemic sclerosis. Indian J Dent Res 2007;18:27-30

How to cite this URL:
Ahathya R S, Deepalakshmi D, Emmadi P. Systemic sclerosis. Indian J Dent Res [serial online] 2007 [cited 2019 Nov 22];18:27-30. Available from: http://www.ijdr.in/text.asp?2007/18/1/27/30919

   Introduction Top


Systemic sclerosis is characterized by progressive fibrosis of skin and multiple organs. The term "scleroderma" literally means 'hard skin' (sclera - hard; derma - skin). Though the term scleroderma is indelibly etched in the literature through common usage, the disease is currently called as "systemic sclerosis". Since hidebound skin is the clinical hallmark of the disease, it is also called "hidebound disease".

Earlier genetic, environmental, autoimmune, vascular, nervous factors were all proposed to be involved in the etiology of the disease.[1],[2],[3] But later on only vascular and environmental factors are found to play a major role in the isolated cases of this disease.

In the early phase of this condition, there is skin infiltration by T lymphocytes and abnormal fibroblast activation, which leads to increased production of extracellular matrix in the dermis, primarily type I collagen.[4] This results in symmetrical thickening, tightening and induration of the skin, which is the clinical scenario of this condition. There is also arterial and arteriolar narrowing due to intimal proliferation and vessel wall inflammation.[5]

The disease has been classified into two major categories.[6] One is the diffuse form in which there is generalized skin involvement and rapid progressive internal organ involvement. The other form is with limited cutaneous involvement confined to the distal aspects of the fingers and the face.

A variant of this disease is known as the "CREST syndrome",[7] which is an acronym for Calcinosis cutis, Raynauds phenomenon, Esophageal dysmotility with dysphagia, Sclerodactyly and Telangiectases.


   Case Report Top


A 26-year- old female patient reported with a complaint of dirty teeth. Her past medical history revealed that she had undergone treatment for white lesions on her forehead two months ago.

On examination, the patient had multiple irregular ill-defined white patches on the forehead, post auricular region [Figure - 1] and on the distal aspect of the hand. Facial skin was smooth taut and mask-like. It was firm and could not be picked up. Stiffening of phalangeal joints in both fingers and toes was observed. Nasal alae was atrophied giving a pinched appearance to the nose, classically known as "mouse facies" [Figure - 1]. Flexure contracture producing shortened 'claw-like' fingers was also present [Figure - 2]. Temperomandibular joint was stiff on palpation.

Oral manifestations included microstomia with characteristic furrows radiating from the mouth, giving rise to 'purse string appearance' [Figure - 3]. Crenations in the buccal mucosa were more prominent [Figure - 4]. Marginal gingiva was firm, red and inflamed in relation to lower anteriors. Periodontal examination revealed generalized mild chronic periodontitis, with probing depth ranging from 4-6 mm in posteriors and 3-5 mm in anteriors.

Radiological examination of hand revealed resorption of the terminal phalanges (acroosteolysis) [Figure - 5],[Figure - 6]. Widening of periodontal ligament space was present in all the teeth, more pronounced in posteriors classically seen in OPG [Figure - 7] and IOPA [Figure - 8],[Figure - 9].

Initial therapy consisted of oral hygiene instructions, scaling and root planning by quadrants and reevaluation. The periodontal pockets responded well to scaling and root planning and improved oral hygiene and hence required no further treatment.


   Discussion Top


Systemic sclerosis is a relatively uncommon condition with an average annual incidence of 6-12 patients per million population.[8] The disease is most prevalent between 30 and 50 years of age. Rarely begins during infancy or after 60 years of age.[9] Females are affected seven times as often as males.[10]

A plethora of signs and symptoms are observed. But hidebound skin is the hallmark of this condition and organ involvement or compromise is the prognostic keystone.[11]

It usually begins on the face and hands with development of indurated edema of skin and later fixation of epidermis to the deeper subcutaneous tissues. Neuralgia and paresthesia may occur. Flexural contractures and resorption of terminal phalanges occur frequently giving rise to 'claw like appearance' of the fingers.

Localized type of scleroderma may occur either as circumscribed morphea form or as linear encoup de sabre form.[12] In the former type, there are presence of white/yellowish cutaneous patches surrounded by violaceous halo of varying size and shape. In the later form, linear ribbons that resemble marks produced by blow of a saber are seen. Lesions are mostly asymptomatic.

Maxillofacial and oral manifestations[10],[13] include loss of skin folds around mouth giving rise to 'mask-like appearance' of the face, atrophied nasal alae giving rise to mouse facies. Crenations are seen along the buccal mucosa with severe buccal mucosal fibrosis. There may also be rigidity of tongue and lips. Eversole et al reported mucogingival periodontal problems, including loss of attached gingiva and multiple areas of gingival recession.[14] The authors attributed the mucogingival changes to strictures associated with sclerodermatous changes in the mandibular mucobuccal fold. Reduced opening of mouth and fixation of jaw are results of involvement of peritemporomandibular joint tissues. Pathological changes of minor salivary glands mimicking Sjogren's disease may also be present. Trigeminal neuropathy consisting of paresthesia / hypesthesia and/ or pain is encountered in 4% of cases with systemic sclerosis.[15]

In diffuse form of systemic sclerosis, fibrosis may occur in internal organs with loss of smooth muscle and loss of visceral function.[12] GIT is affected in more than 50% of cases, mostly affecting distal esophagus causing dysphagia. Lung disease ranks second to GIT in the frequency of visceral involvement. Dyspnoea and hypoxia may result from interstitial inflammation and fibrosis. Kidney when affected has highest mortality and cardiac involvement may result in cardiac failure. Soft tissue calcifications may also be seen.

Regarding roentgenographic features, most studies in the dental literature have focused on widened periodontal ligament changes and mandibular angle osseous resorption.[13],[16],[17] Extreme widening of periodontal ligament i.e., two to four times the normal thickness is a characteristic feature of scleroderma. It occurs in about 10-37% of cases.[13] Periodontal ligament of posterior teeth has most pronounced alterations.[13]

Mandibular resorption in scleroderma was first reported by Taveras in 1959.[18] Resorption resembling " Gorhams Massive Osteolysis" [19] is due to excessive involvement of facial tissues and muscles.[10] Resorption of the terminal phalanges of the hand and the distal portions of the radius and ulna are the most frequent radiologic findings in scleroderma patients.[20]

Diagnosis is mostly by these hallmark clinical and roentgenographic features. Non-specific serological abnormalities such as hyperglobulinemia, anti nuclear antibodies (70-90% of cases) and rheumatoid factor (25% of cases) may also be present.

Prognosis among Caucasians is worse in men than in women, but it tends to be more severe in blacks especially in black females. Patients with mainly skin involvement have a more gradual and favorable course than those with visceral disease.[21] Overall five years survival rate is approximately 70%.[5]

No agent has demonstrated efficacy in arresting / improving skin changes. Self-management to maintain core body temperature and avoid peripheral cold exposure is important. Infections of the ulcerated skin should be treated with prompt antibiotic therapy.[5]

Dental treatment does not pose any risk for these patients. However, alterations in patient management are necessary. Stretching exercises, use of increased number of tongue blades between posterior teeth to stretch facial tissues and bilateral commissurotomy are done to manage these patients.

The preventive nature of proper oral hygiene habits and the need for good permanent restorative dentistry should be stressed to these patients because the progressive constriction of the oral cavity eventually limits access to perform adequate dental treatment.

 
   References Top

1.Korn JH. Immunologic aspects of scleroderma. Curr Opin Rheumatol 1990;2:922-8.  Back to cited text no. 1  [PUBMED]  
2.Welsh KL, Briggs DC. Genetic and environmental factors in scleroderma. Curr Opin Rheumatol 1990;2:920-1.  Back to cited text no. 2    
3.Wilson RH, McCormick WE, Tatum CF, Creech JL. Occupational acroosteolysis. Report of 31 cases. J Am Med Assn 1967;201:577-81.  Back to cited text no. 3    
4.Leroy EC. Connective tissue synthesis by scleroderma skin fibroblasts in cell culture. J Exp Med 1972;135:1351-62.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]
5.Haslett C, Chilvers ER, Boon NA, Coolidge NR. Davidson's principles and practice of medicine. 19th ed. p. 1036-7.  Back to cited text no. 5    
6.Masi AT. Classification of systemic sclerosis (scleroderma): Relationship of cutaneous subgroups in early disease to outcome and serologic reactivity. J Rheumatol 1988;15:894-8.  Back to cited text no. 6  [PUBMED]  
7.Veloyos EE, Masi AT, Stevens MB, Shulman LE. The CREST syndrome: Comparison with systemic sclerosis (Scleroderma). Arch Intern Med 1979;139:1240-5.  Back to cited text no. 7    
8.Medsger TA Jr, Masi AT. Epidemiology of systemic sclerosis (scleroderma). Ann Intern Med 1972;74:714-21.  Back to cited text no. 8    
9.Haers PE, Sailer HF. Mandibular resorption due to systemic sclerosis. Case report of surgical correction of a secondary open bite deformity. Int J Oral Maillofac Surg 1995;24:261-7.  Back to cited text no. 9    
10.Wardrop RW, Heggie AA. Progressive systemic sclerosis-Oro-facial manifestations. Case report. Aust Dent J 1987;32:258-62.  Back to cited text no. 10    
11.Leroy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger TA Jr, et al . Scleroderma (systemic sclerosis): Classification, subsets and pathogenesis. J Rheumatol 1988;15:202-5.  Back to cited text no. 11  [PUBMED]  
12.Gonzales TS, Coleman GC. Periodontal manifestations of collagen vascular disorders. Periodontol 2000 1999;21:94-105.  Back to cited text no. 12    
13.Marmary Y, Glaiss R, Pisanty S. Scleroderma - Oral manifestations. Oral Surg Oral Med Oral Pathol 1981;52:32-7.  Back to cited text no. 13  [PUBMED]  
14.Eversole LR, Jacobson PL, Stones CE. Oral and gingival changes in Systemic sclerosis (Scleroderma). J Periodontol 1984;55:175-8.  Back to cited text no. 14    
15.Farrell DA, Medsger TA Jr. Trigemial neuropathy in progressive systemic sclerosis. Am J Med 1982;73:57-62.  Back to cited text no. 15  [PUBMED]  [FULLTEXT]
16.Stafne EC. Dental roentgenologic manifestations of systemic disease. III. Granulomatous disease, Paget's disease, acrosclerosis and others. Radiology 1952;58:820-9.  Back to cited text no. 16    
17.White SC, Frey NW, Blaschke DD, Ross MD, Clements PJ, Furst DE, et al . Oral radiographic changes in patients with progressive systemic sclerosis (Scleroderma). J Am Dent Assoc 1977;94:1178-82.  Back to cited text no. 17  [PUBMED]  
18.Taveras JM. The interpretation of radiographic disorders of the TMJ. N R Saunders: Philadelphia; 1959. p. 154-62.  Back to cited text no. 18    
19.Heffez L, Doku HC, Carter BL, Feeney JE. Perspectives on massive osteolysis. Report of a case and review of the literature. Oral Surg Oral Med Oral Pathol 1983;55:331-43.  Back to cited text no. 19    
20.LeRoy EC. Sentinal signs and symptoms of systemic sclerosis. Curr Opin Rheumatol 1990;2:942-6.  Back to cited text no. 20  [PUBMED]  
21.Altman RD, Medsgar TA Jr, Bloch DA, Micher BA. Predictors of survival in Systemic sclerosis. Arthritis Rheum 1991;34:403-13.  Back to cited text no. 21    

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Correspondence Address:
R S Ahathya
Department of Periodontics, Meenakshi Ammal Dental College and Hospital, Alapakkam Main Road, Maduravoyal, Chennai - 600 095
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0970-9290.30919

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    Figures

[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7], [Figure - 8], [Figure - 9]

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