Scleroderma and dentistry: Two case reports

Abstract

BackgroundScleroderma 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 presentationCase 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.ConclusionThrough 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.

Progressive Hemifacial Atrophy and Linear Scleroderma En Coup de Sabre: A Spectrum of the Same Disease?

“…The investigation indicated a close relationship between PHA and ECDS. Dixit and colleagues presented a case of mucosal involvement in morphea, associated with PHA ( 122 ). The development or transition of ECDS into PHA in the same physical location was described as well ( 6 , 123 ).…”

Characterization of dental phenotypes and treatment modalities in Korean patients with Parry–Romberg syndrome

“…We summarized the dental phenotypes in our PRS patients and compared our findings with those of the previous case reports and review (Table 5). 8,10,11,[13][14][15][16][18][19][20][21] First, congenitally missing teeth were reported by Urban et al 16 and Grippaudo et al 20 in their case reports ( Table 5). In the present study, the prevalence of congenital missing teeth was 60%, and the side of occurrence matched with the side of PRS involvement in all six patients (Table 2).…”

“…[2][3][4][8][9][10] Therefore, a multidisciplinary treatment protocol involving dermatologists, surgeons, dentists, and psychologists should be applied to correct the soft tissue deformities and reconstruct the skeletal framework. 11,12 There have been numerous case reports or reviews of a single PRS case or cases exhibiting diverse dental phenotypes (congenitally missing teeth, delayed eruption/impaction, abnormal crown and root morphology, or malocclusion) 8,10,[13][14][15][16][17][18][19] and treated by orthodontic approaches. 11,20,21 However, these studies did not show the exact entity of the dental phenotypes, treatment modality (Tx-Mod) and its timing, and course of longterm treatment.…”

Systemic sclerosis and its oral health implications

“…17 In severe cases, surgical procedures (e.g., bilateral commissurotomy or commissuroplasty) are indicated for the increase in mouth opening. 18 Dermatological treatments (such as photodynamic therapy, phototherapy with UV long wavelength light or photochemotherapy) demonstrate benefits. 16 Comstedt et al in their pilot study, treated SSc patients with microstomia using intense pulse light (IPL), which is known for its ability to remodulate the collagen formation in the skin.…”