Limited systemic sclerosis
Overview
Systemic sclerosis (SSc) is a systemic connective tissue disease. Characteristics of SSc include essential vasomotor disturbances; fibrosis; subsequent atrophy of the skin, subcutaneous tissue, muscles, and internal organs (eg, alimentary tract, lungs, heart, kidney, CNS); and immunologic disturbances accompany these findings.
Symptoms
Calcinosis of skin (40%) Raynaud's phenomena (90-95%) oesophageal involvement (90%) resulting in progressive dysphagia and dyspepsia Sclerodactyly (90%) tight shiny appearance of skin on fingers and toes, skin changes may also occur in bands giving the classic "coup de sabre" appearance. Telangiectasia (50%) on face, lips tongue, chest wall etc.
Diagnosis
Features of the disease are divided into 2 groups: Major features include centrally located skin sclerosis that affects the arms, face, and/or neck. Minor features include sclerodactyly, erosions, atrophia of the fingertips, and bilateral lung fibrosis. Diagnosis of systemic sclerosis is made when a patient has 1 major and 2 minor criteria.3
Treatment
Nodules of calcinosis may require surgical removal. Skin ulcers, if severe, may require skin grafts. Contractures may require surgical release.