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IJHSR. 2016; 6(12): 329-334

Progressive Systemic Sclerosis: A Case Report and Review of Literature.

Nirupa Thomas, Thomas Priya, Rekha Krishna Pillai, Ramakrishnan Bindhu P.

Systemic sclerosis (SSc) is a collagen disorder, characterized by vasomotor disturbances, fibrosis, and atrophy of skin, subcutaneous tissue, muscles, and internal organs (alimentary tract, lungs, heart, kidney, CNS) with associated immunologic disturbances. Dense collagen is deposited in the tissues of the body in extraordinary amounts. Systemic sclerosis can affect the facial and oral structures as well and can present as diagnostic dilemma. This paper reports a case of systemic sclerosis in a 24 year old female patient with hard, shiny skin, depigmentations, mouse facies, resorption of terminal phalanges, claw like hands, microstomia, reduced mouth opening, and fibrosis of oral mucosa. Orthopantomogram showed generalized PDL space widening and resorption of condyle. The characteristic presentations in this case enabled us to establish the diagnosis of progressive systemic sclerosis.

Key words: systemic sclerosis, collagen disorder, microstomia.

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