Scleroderma: a case report.

Scleroderma is an autoimmune connective tissue disorder which is characterized by fibrosis of visceral organs, skin and blood vessels. This condition can be localized or systemic. Its estimated prevalence is 250 cases in a million and it is more common in women than in men. Resorption of the mandibular angle and coronoid process can be observed in patients with scleroderma. Pressure of fibrous mucocutaneous tissues is thought to be the cause of the resorption. Decreased number of wrinkles due to sclerosis and distinct facial features because of the atrophy of ala nasi are among common clinical characteristics of this condition. The aim of this case report is to present a 40-year-old female patient with scleroderma who presented with signs of resorption at the angle of mandible, coronoid process, as well as widening of the periodontal space.

Introduction

The word scleroderma originates from Greek words “scleros” meaning hard and “derma” which means skin (1). Scleroderma is a connective tissue disorder which is characterized by fibrosis of the skin, internal organs and blood vessels. It is categorized into two subgroups which are described as systemic and localized. Systemic sclerosis differs from localized scleroderma because it is accompanied by Raynaud’s phenomenon, acrosclerosis and internal organ involvement (2, 3, 4). Normal collagen type I and III are thought to be deposited excessively at various tissues by immunologically over-activated fibroblasts (1).

Although the etiology of scleroderma is not clear, genetic and environmental factors have been held responsible for the onset of this condition. First degree relatives of scleroderma patients have a high probability of developing the disease. Silica powder, vinyl chloride, L-tryptophan, silicone breast implants and organic solvents are among the environmental factors which were suspected to cause this condition (4). Scleroderma is mostly seen in women whose ages are between 30 and 50 and its prevalence is 250 cases in a million (2, 5).

Scleroderma causes various changes at the oral and facial tissues both clinically and radiographically. Radiographic changes in scleroderma patients are the widening of periodontal space, loss of lamina dura, and resorption of the mandibular angle (6). The first specific clinical sign of scleroderma is the swelling on the skin of hands and fingers (5). The aim of this case report is to present some clinical and radiographic characteristics of a patient diagnosed as having scleroderma.

Case Report

A 40-year-old female patient was referred to Oral and Maxillofacial Radiology Department, Istanbul University Faculty of Dentistry for dental complaints. Her medical history and the documentation she presented, revealed that the patient was diagnosed with scleroderma and she had been treated in the Department of Rheumatology at Istanbul University Faculty of Medicine, for 20 years. She had goiter and gastric disease. Extra-oral examination showed that the patient was not able to open her hands because of sclerosis (Figure 1) and had a narrow nose because of atrophy of ala nasi (Figure 2).

Figure 1.

Patient’s fingers which were fixed at claw-like position.

Figure 2.

Facial expression and insufficient upper lip cover.

Decreased number of wrinkles were also observed. Upper lip was insufficient to cover maxillary teeth, which is also known as “mouse-like face”. Anamnesis revealed that the patient had Raynaud phenomenon. She had used cortizon and acetylsalicylic acid for twenty years due to the vascular decomposition. Although Raynaud’s phenomenon and acrosclerosis were present in our case, there was no internal organ involvement. Patient complained about the dryness of her mouth and multiple caries lesions were observed. Trismus and increased overjet were observed in the intra-oral examination (Figure 3). Panoramic radiography disclosed bone resorption in the right mandibular angle region of the mandible and in both ramus (Figure 4. The patient was referred to the departments for specialized dental treatment.

Figure 3.

Patient’s maximum mouth opening.

Figure 4.

Panoramic radiography of the patient.

Discussion

Scleroderma is an autoimmune disease in which the antibodies target blood vessels and connective tissues (5). There is no consensus on the treatment protocol (2, 4, 5). Although being controversial, penicillamine has been used to inhibit collagen deposition. Corticosteroids have no effect on the progression of the disease (4, 7). Other treatment options are based on controlling the symptoms (7). Treatment for scleroderma is selected and organized according to the affected organs (4).

Resorption of the terminal phalanxes, short and claw-like fingers because of acro-osteolisis, ulcers on finger tips are common in patients with scleroderma (7, 8). About 80% of them present with symptoms in the head and neck region (8). Dysphagia and gastroesophageal reflux are frequently reported complaints in this group of patients. Trismus, widening of the periodontal space, decrease in facial wrinkles owing to fibrosis of skin, orofacial telengiectasia, resorption of mandibular angle are some of the changes which may occur in the oral and maxillofacial region. Xerostomia and increased frequency of dental caries might be seen due to the secondary Sjögren syndrome (1, 2, 8). Rarely, mandibular resorption can lead to pathologic fractures (1). Although xerostomia is not present, high frequency of dental caries is attributed to inadequate oral hygiene by virtue of sclerosis of hands. Resorptions at the right mandibular angle, coronoid process and ramus region were present in our case and the patient’s oral hygiene was poor.

Excessive collagen deposition in the cutaneous tissues around the mouth causes microstomia which prevents the patient from opening and closing the mouth. Fibrosis of salivary glands leads to xerostomia, dysphagia, and subsequently periodontal infections (1, 5). Although microstomia and severe periodontitis were present in our case, xerostomia and dysphagia were not observed. Radiographic changes in scleroderma patients are widening of the periodontal space, loss of lamina dura, resorption of the mandibular angle, zygomatic arch, digastric region, caput mandible, and coronoid process. Blunting of mandibular angles leads to radiographic features which are described as “tail of the whale” (1). White et al. (6) reported widening of the periodontal space in 37% of the scleroderma patients. Wood et al. (9) observed mandibular resorption in 29% of the patients. Excessive deposition of collagen and oxytalan fibers and the resorption of the alveolar crest around tooth roots might cause periodontal widening (9, 10). In our patient, periodontal widening, tail of the whale appearance, and mandibular resorption were present.

Conclusion

Scleroderma is a systemic disease which causes significant radiographic and clinical changes in the oral and maxillofacial structures. Dental professionals must be aware of these changes since xerostomia and limited mouth opening are especially important for the right diagnosis and treatment. Multidisciplinary approach is of utmost importance for the scleroderma patients because of the complications of disease.