title: 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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors: Department of Oral Medicine and Radiology, Dhulikhel hospital, Kathmandu University School of Medical Sciences, Dhulikhel, Nepal. drshantanudixit@gmail.com.
authors: Shantanu
authors: S
authors: Dixit
authors: Department of Oral Medicine and Radiology, Century International Institute of Dental Science and Research Centre, Kasargod, Kerala, India.
authors: Chaithra
authors: C
authors: Kalkur
authors: Department of Oral Medicine and Radiology, SDM College of Dental Sciences, Sattur, Karnataka, India.
authors: Atul P
authors: AP
authors: Sattur
authors: Department of Oral Surgery and Stomatology, School of Dental Medicine, University of Bern, Bern, Switzerland.
authors: Michael M
authors: MM
authors: Bornstein
authors: General Practitioner, Wenatchee, Washington, USA.
authors: Fred
authors: F
authors: Melton
mesh_terms: D003813:Dentistry; D005150:Facial Hemiatrophy / Q000175:diagnosis* / Q000473:pathology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010808:Physical Examination; D035583:Rare Diseases; D012017:Referral and Consultation; D012219:Rheumatology; D012594:Scleroderma, Localized / Q000175:diagnosis* / Q000473:pathology; D055815:Young Adult
keywords: CREST syndrome; Localized scleroderma; Morphea; Parry–Romberg syndrome; Raynaud’s phenomenon; Systemic sclerosis