Progressive systemic sclerosis with intraoral manifestations: A case report and review

Abstract

The word scleroderma comes from two Greek words, “sclero” meaning hard and “derma” meaning skin. Scleroderma or progressive systemic sclerosis (PSS), a rare condition, was first characterized as a single condition in 1752 by Curzio of Naples. It generally affects woman between 30 and 50 years of age and has a low prevalence. Scleroderma is a disease of the immune system, blood vessels, and connective tissue. Dermal manifestations include stiff, tight, and shiny skin usually of the hands and feet due to swelling and thickening of the connective tissue as they become fibrotic or scarred. Other symptoms include difficulty in swallowing, bloating, abdominal pain, tiredness, lack of energy, weight loss, aching muscles, joints, and bones. The vital organs that may get involved are lungs, heart, and kidneys. We present a case report of PSS in a 45-year-old female patient with characteristic systemic and oral manifestations.

Periodontal Diseases and Lipid Profile in Systemic Sclerosis: an EUSTAR Cohort Experience

“…Orofacial manifestations are commonly described among SSc patients (more than two thirds of cases) and, apparently, multifactorial [1][2][3][4][5][6]. Recent studies focused on severe impaired oral health indicating several SSc-related specific factors such as decreased oral aperture (microstomia) (43% to 80% cases) with abnormal interincisal distance (mean inter-incisal distance for people with SSc being about 33 mm), excessive dry mouth (decreased salivary flow secondary to concomitant Sjogren's syndrome) (up to 60% cases), gastroesophageal reflux disease, reduced manual dexterity (hand deformity, contractures) to maintain optimal oral hygiene, defective vascularity and alterations of the microcirculation of the gingival tissues with subsequent gingival inflammation [1][2][3][4][5][6][7][8][9]. Moreover, immunosuppressant drugs and symptomatic medication e.g.…”

“…It is widely accepted that dental pathology comprises more missing teeth, tooth decay and more periodontal disease in SSc as compared to age-and sex-matched healthy controls [1][2][3][4][5][6][7][8][9]. Furthermore, patients are at increased risk for dental plaque, gingival hyperplasia, gingival inflammation and, even, gingival bleeding (about 60%) [9 -11].…”

“…Although the orofacial manifestations are typically reported in SSc, only few studies have adequately addressed the issue of oral health-related quality of life and derived dental pathology in such pathobiological settings [1][2][3][4][5][6][7][8][9][10][11].…”

Caracterização do frênulo e dos aspectos da língua de indivíduos com esclerose sistêmica

“…Nesta afecção, as mulheres são acometidas de três a quatro vezes mais que os homens e a idade média de aparecimento da doença ocorre aos 50 anos 15 , geralmente entre os 30 e 50 anos 16 ratificando os resultados obtidos neste estudo, como visualizado na tabela 1.…”

Comment on “Facial Atrophy in Oral Submucous Fibrosis: An Association or a Coincidence”

“…It is characterized by enormous deposition of collagen, alterations of the microvasculature, and disturbances of the immune system [2]. Oral manifestations in SS are very common and these include limited ability to open the mouth, facial and tongue rigidity, thinning of lips, xerostomia, periodontal disease, increased periodontal ligament width, and osseous resorption of the mandible [2, 3]. A few decades back, plenty of cases have been reported with neurological manifestations as well [4, 5].…”