Scleroderma and dentistry: Two case reports

Abstract

Background: Scleroderma is a chronic connective tissue disorder with unknown etiology. It is characterized by excessive deposition of extracellular matrix in the connective tissues causing vascular disturbances which can result in tissue hypoxia. These changes are manifested as atrophy of the skin and/or mucosa, subcutaneous tissue, muscles, and internal organs. Such changes can be classified into two types, namely, morphea (localized) and diffuse (systemic). Morphea can manifest itself as hemifacial atrophy (Parry-Romberg syndrome) although this remains debatable. Hence, we present a case of morphea, associated with Parry-Romberg syndrome, and a second case with the classical signs of progressive systemic sclerosis.

Case presentation: Case one: A 20-year-old man of Dravidian origin presented to our out-patient department with a complaint of facial asymmetry, difficulty in speech, and loss of taste sensation over the last 2 years. There was no history of facial trauma. After physical and radiological investigations, we found gross asymmetry of the left side of his face, a scar on his chin, tongue atrophy, relative microdontia, thinning of the ramus/body of his mandible, and sclerotic lesions on his trunk. Serological investigations were positive for antinuclear antibody for double-stranded deoxyribonucleic acid and mitochondria. A biopsy was suggestive of morphea. Hence, our final diagnosis was mixed morphea with Parry-Romberg syndrome. Case two: A 53-year-old woman of Dravidian origin presented to our out-patient department with a complaint of gradually decreasing mouth opening over the past 7 years. Her medical history was noncontributory. On clinical examination, we found her perioral, neck, and hand skin to be sclerotic. Also, her fingers exhibited bilateral telangiectasia. An oral examination revealed completely edentulous arches as well as xerostomia and candidiasis. Her serological reports were positive for antinuclear antibodies against centromere B, Scl-70, and Ro-52. A hand and wrist radiograph revealed acro-osteolysis of the middle finger on her right hand. Hence, our final diagnosis was progressive systemic sclerosis.

Conclusion: Through this article, we have tried to emphasize the importance of a general examination when diagnosing rare systemic diseases such as scleroderma and the role of the general dentist when caring for such patients, even though they can be quite rare in general practice.

Keywords: CREST syndrome; Localized scleroderma; Morphea; Parry–Romberg syndrome; Raynaud’s phenomenon; Systemic sclerosis.

Fig. 1

Morphea seen on trunk (back) of patient

Fig. 2

Gross asymmetry of face (left side) with “en coup de sabre” on chin

Fig. 3

Atrophy of tongue (left side)

Fig. 4

Panoramic view showing gross asymmetry of face (left side) with relative microdontia and thinning of ramus/body of mandible

Fig. 5

Microstomia resulting in difficult prosthesis placement

Fig. 6

Shiny and tense skin of hand showing Raynaud’s phenomenon and telangiectasia (encircled)

Fig. 7

Hand and Wrist radiograph revealing acro-osteolysis (encircled) of terminal phalanges of right middle finger