Limited Mouth Opening Secondary to Diffuse Systemic Sclerosis

Systemic sclerosis (SSc) is a relatively rare condition with an immunologically mediated pathogenesis. For reasons that are not clearly understood, dense collagen is deposited in the connective tissues of the body in extraordinary amounts. Although its dramatic effects are seen in association with the skin, the disease is often quite serious with visceral organ involvement. We describe a case of limited mouth opening secondary to diffuse SSc, improvement in mouth opening with passive jaw stretch exercises, and the challenges involved in performing dental procedures for such patients. 1. Introduction Systemic sclerosis (SSc) or scleroderma is an incurable and potentially life-threatening systemic autoimmune connective tissue disease of unknown etiology and multifactorial pathogenesis characterized by cutaneous and visceral fibrosis, microvascular obliteration, and highly specific serum autoantibodies to nuclear autoantigens [1, 2]. SSc is divided into diffuse cutaneous, and limited cutaneous forms based on the extent of skin involvement. Various genetic, infectious and environmental factors have been implicated in the etiopathogenesis of SSc [3]. Orofacial tissue involvement is a typical feature in patients suffering from SSc; the subject’s face becomes expressionless, the cutaneous furrows disappear, and the nose becomes sharp. The alterations in facial form also include decreased mouth opening (microstomia). This may interfere considerably with eating, speaking, oral hygiene measures, and dental treatment, thus deteriorating the quality of life of these subjects [4, 5]. This paper describes a case of limited mouth opening secondary to diffuse SSc, improvement in mouth opening with passive jaw stretch exercises, and the challenges involved in performing dental procedures for such patients. 2. Case Report A 22-year-old African American woman reported to Orofacial Pain and Oral Medicine Center at the Ostrow School of Dentistry, University of Southern California, with a complaint of limited mouth opening. The patient had been referred by her general dentist for evaluation and management of her limited mouth opening. Examination of the patient revealed a narrow mask-like face, with taut facial and cervical skin (Figure 1(a)). Her blood test was positive for Anti-Scl70 (topoisomerase I) antibodies typical of diffuse SSc. The patient’s maximum mouth opening was 10？mm with a hard end feel on passive stretch. Examination of the hands revealed deformed fingers with taut skin indicative of sclerodactyly (Figure 1(b)). Her medical history was significant for