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 Table of Contents  
CASE REPORT
Year : 2014  |  Volume : 26  |  Issue : 1  |  Page : 115-118

Localized scleroderma: A rare clinical entity


Department of Oral Medicine and Radiology, Seema Dental College and Hospital, Rishikesh, Uttarakhand, India

Date of Submission22-May-2014
Date of Acceptance10-Sep-2014
Date of Web Publication26-Sep-2014

Correspondence Address:
Ranjana Garg
Seema Dental College and Hospital, Rishikesh - 249 203, Uttarakhand
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-1363.141877

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   Abstract 

Localized Scleroderma (LS), which is also referred to as morphea, is an inflammatory disease that leads to diffuse or localized fibrotic and atrophic skin hardening. It is an uncommon, autoimmune, multisystem connective tissue disease that is characterized by excessive collagen deposition, widespread microvascular abnormalities, widespread skin fibrosis, and proliferative obstructive vasculopathy, which are the hallmark pathological features. Here we present a case of localized scleroderma in a middle-aged female patient.

Keywords: Autoimmune disorder, fibrosis, scleroderma


How to cite this article:
Garg R, Dhingra S, Arora R. Localized scleroderma: A rare clinical entity . J Indian Acad Oral Med Radiol 2014;26:115-8

How to cite this URL:
Garg R, Dhingra S, Arora R. Localized scleroderma: A rare clinical entity . J Indian Acad Oral Med Radiol [serial online] 2014 [cited 2019 Sep 18];26:115-8. Available from: http://www.jiaomr.in/text.asp?2014/26/1/115/141877


   Introduction Top


Scleroderma is an autoimmune rheumatic condition affecting the connective tissue, which is characterized by fibrosis that involves the skin, muscle, and organ systems. It was first named by Goetz in 1945, as 'progressive systemic sclerosis' (PSS). It occurs commonly in women and the age of peak incidence is between 30 and 50 years. The disease can occur both in systemic or localized forms. Its oral manifestations include features such as, restricted mouth opening, widening of the periodontal ligament space, pseudo-ankylosis, malocclusion, mandibular resorption and premature loss of teeth. This case report attempts to correlate these findings, which will enable the clinician to give a correct diagnosis and evolve a proper treatment plan for individual patients. [1]


   Case Report Top


A patient aged 38 years, reported to the Department of Oral Medicine and Radiology, with a chief complaint of missing teeth and wanted an artificial replacement for the same, as she was unable to chew food. The patient gave a history of mobility in relation to all her teeth for the past two years that had eventually led to loss of all her teeth.

There was no history of any deleterious habits. The patient's past medical and dental history was insignificant. There were no systemic abnormalities and all the vital signs were within the normal range. The patient was unable to straighten the little finger of her right hand. The skin on the hands and legs was stiff, with reduced flexibility [Figure 1]a-c.
Figure 1: (a) Loss of elasticity of the skin over the legs; (b) patient unable to straighten her little finger due to stiffness of the skin; (c) stiffness of the skin over the hands

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On extraoral examination, her face showed a mask-like appearance, due to the absence of the normal creases. The nose appeared to be mouse-like, with a pinched appearance and atrophied nasal alae [Figure 2]. A small opening of the mouth (microstomia), due to extensive perioral fibrosis, was appreciated, with features of reduced elasticity of the skin over the face [Figure 3]a. Stiffness of the cartilage of the ear was also seen. However, there was no significant obvious facial asymmetry.
Figure 2: Face showing a mask-like appearance with mouse facies

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Figure 3: (a) Microstomia; (b) depapillation over the dorsal surface of the tongue; (c) missing Uvula; (d) brownish pigmentation seen on the ventral surface of the tongue and floor of the mouth

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On intraoral examination, blanching was seen on the floor of the mouth with restricted tongue movements. Areas of depapillation were appreciable over the dorsal surface of the tongue [Figure 3]b. The uvula of the patient was missing and there was an oropharyngeal communication [Figure 3]c. Brownish pigmentation was seen over the ventral surface of the tongue [Figure 3]d. The buccal mucosa had palpable vertical bands. There was reduced salivary flow from the ducts of the major salivary glands. Considering the reduced mouth opening and other intraoral findings of the patient, a provisional diagnosis of oral submucous fibrosis was made. The orthopantomograph revealed reduced vertical height of the mandible, with alveolar bone resorption. Resorption was even seen at the right and left angles of the mandible and the antegonial notch was prominent on both the sides [Figure 4]. On the basis of the clinical and radiographic findings, a diagnosis of localized scleroderma was made and the patient was referred for further systemic evaluation.
Figure 4: OPG showing resorption at the angle of mandible and a prominent antegonial notch bilaterally

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   Discussion Top


Scleroderma originates from the Greek words skleros, meaning 'hard,' and derma, meaning 'skin'. Scleroderma is the initial manifestation of a disease process better described as progressive systemic sclerosis (PSS), which was named by Goetz, in 1945. [2]

Scleroderma is a chronic inflammatory disease of unknown origin and autoimmune nature, characterized by excessive deposition of collagen and glycosaminoglycans in the conjunctive tissue of the dermis and internal organs. [1]

Progressive tissue fibrosis ensues, as type I and III collagen are deposited in extraordinary amounts, most likely due to the immunologically overactivated fibroblasts in various locations. There is a quantitative increase in the amorphous ground substance, glycosaminoglycans, and fibronectin, within the connective tissues. Vascular alterations may affect the small arteries and arterioles, resulting in thickening of the vessel walls and reduction of the diameter of the lumen. Vascular dysfunction is one of the earliest alterations found in those with PSS, and may represent the initiating event in its pathogenesis, similar to that of many other rheumatic diseases. [3]

The disease can present in two forms, systemic or localized. The localized form of the disease can present as morphea or en coup de sabre, which differs from systemic sclerosis by the absence of systemic involvement. Morphea or circumscribed scleroderma is characterized by local changes limited to the skin, which assume a thickened, 'hidebound' character. [4] In addition, localized scleroderma is characterized by the absence of the following findings: Raynaud phenomenon, digital sclerosis, digital necrosis, nailfold capillary changes, and involvement of internal organs. [5]

It is a disease of low incidence, with an average of 4-19 new cases per million inhabitants and preferentially it affects females (4:1). [1] The age group most affected is between the third and fifth decades of life. [6]

The conjunctival tissue undergoes fibrosis, which is probably mediated by cytokines produced by lymphocytes and inflammatory cells, particularly the transforming growth factor beta (TGF-β). The microcirculation undergoes a process of primary vasculitis, which in conjunction with the differing vessel thicknesses may cause total obliteration of the vessel due to collagen deposition, thereby leading to clinical manifestations of varying degrees of severity. [7]

The skin develops a diffuse, hard texture, which is difficult to pinch, and its surface is usually smooth and taut, with mask-like facies. Similarly, the nasal alae become atrophied, resulting in a pinched appearance of the nose, called as mouse facies. Skin over the extremities, face, and trunk may be with dark pigmentation and contrasting areas of hypopigmentation may also develop. The sparing of pigment around the hair follicles gives the skin a 'salt and pepper' appearance. [8]

The oral manifestations include microstomia, xerostomia, telengiectasias, and multiple decayed or missing teeth. The tongue can also become rigid, making speech and swallowing difficult. [9] Furthermore, facial and mucosal fibrosis compromises oral access because of microstomia, which limits the opening of the mouth in 70% of these patients. As a consequence, oral hygiene and fabrication of removable dentures are difficult, because of limited access and the obliteration or shallowing of the mucobuccal folds. [10] The case reported here has all the classic features of localized scleroderma.

The radiographic findings include a uniform widening of the periodontal ligament space, especially around the posterior teeth. Also, the mandible shows varying degrees of bone resorption. [9] In the case reported here the patient developed mobility in all her teeth over a period of two years, which eventually resulted in the exfoliation of all the teeth. When the patient reported to the department, she was completely edentulous.

Although much of the fibrosis caused by the disease process of PSS is irreversible, current therapies aim to halt or limit its further progression. Skin sclerosis is often treated with D-penicillamine, a chelating agent that affects unknown mechanisms of collagen formation. Raynaud's phenomenon can be treated with calcium channel blockers, prazosin, and prostaglandin derivatives, such as, prostaglandin E1, aspirin, and topical nitrates.

Patients with limited range of motion and mouth opening can benefit from regular physical and occupational therapy to maintain the range of motion and to minimize or delay contractures. Rarely, patients may benefit from bilateral commissurotomy to increase the width of their mouth. [11]


   Conclusion Top


Scleroderma can give rise to various oral problems, most commonly limited mouth opening. Mouth stretching exercises and facial grimacing are probably the best treatment options for this problem. Good oral care is essential for patients with scleroderma, to keep their mouth free of dental caries and periodontal diseases. It is advised that patients with scleroderma visit a dentist and dental hygienist once in at least three months to ensure the maintenance of good oral health care.

 
   References Top

1.Mayes MD, Lacey JV Jr, Beebe-Dimmer J, Gillespie BW, Cooper B, Laing TJ, et al. Prevalence, incidence, survival and disease characteristics of systemic sclerosis in a large US population. Arthritis Rheum 2003;48:2246-55.  Back to cited text no. 1
    
2.Goetz RH. Pathology of progressive systemic sclerosis (generalized scleroderma) with special reference to changes in the viscera. Clin Proc 1945;4:z337-92.  Back to cited text no. 2
    
3.Kuroda K, Shinkai H. Decorin and glycosaminoglycan synthesis in skin fibroblasts from patients with systemic sclerosis. Arch Dermatol Res 1997;289:481-5.  Back to cited text no. 3
    
4.AuluckA, Pai KM, Shetty C, Shenoi SD. Mandibular resorption in progressive systemic sclerosis: A report of three cases. Dentomaxillofac Radiol 2005;34:384-6.  Back to cited text no. 4
    
5.Schanz S, Fierlbeck G, Ulmer A, Schmalzing M, Kümmerle-Deschner J,Claussen CD, et al. Localized scleroderma: MR findings and clinical features. Radiology 2011;260:817-24.  Back to cited text no. 5
    
6.Spackman GK. Scleroderma: What the general dentist should know. Gen Dent 1999;47:576-9.  Back to cited text no. 6
[PUBMED]    
7.Marcucci M, Abdala N. Clinical and radiographic study of orofacial alterations in patient with systemic sclerosis. Braz Oral Res 2009;23:82-8.  Back to cited text no. 7
    
8.Gilliland BC. Systemic sclerosis (scleroderma). In: Braunwald E, Fauci AS, Kasper DL, Hauser SL, Longo DL, Jameson JL, editors. Harrison's principles of internal medicine. 15 th ed. Vol. 2. New York City, U.S: McGraw-Hill Company; 2001. p. 1941.  Back to cited text no. 8
    
9.Jagadish R, Mehta DS, Jagadish P. Oral and periodontal manifestations associated with systemic sclerosis: A case series and review. J Indian Soc Periodontol 2012;16:271-4.  Back to cited text no. 9
[PUBMED]  Medknow Journal  
10.Neville BW. Dermatologic diseases. In: Neville BW, Damm DD, Allen CM, Bouquot JE, editors. Oral and Maxillofacial Pathology. 2 nd ed. Philadelphia: WB Saunders Co.; 2002: p. 137.  Back to cited text no. 10
    
11.Albilia JB, Lam DK, Blanas N, Clokie CM, Sándor GK.Small mouths … big problems? A review of scleroderma and its oral health implications. J Can Dent Assoc 2007;73:831-6.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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