Ans. is 'a' i.e., Diffuse periosteal reaction Skin involvement in systemic sclerosis Skin involvement is a nearly universal feature of systemic sclerosis (SSc). It is characterized by variable extent and severity of skin. Thickening and hardening. The fingers, hands, and face are generally the earliest areas of the body involved. Edematous swelling and erythema may preceede skin induration. Other prominent skin manifestations include : Pruritus in the early stages Edema in the early stages Sclerodactyly Digital ulcers Pitting at the fingeips Telangiectasia Calcinosis cutis Radiographs of the hands may reveal Soft tissue calcifications (calcinosis cutis). Resorption of the distal phalangeal tufts (acro-osteolysis). Less common radiographic findings are : Aicular erosions Joint space narrowing Demineralization The symptoms of the female and presence of antinuclear antibody points towards the diagnosis of systemic sclerosis. It is a case of systemic sclerosis or scleroderma. The clues to the diagnosis of scleroderma are : Sclerodactyly Raynaud's phenomenon Dysphagia Presence of antinuclear antibody Though systemic sclerosis is a multisystem disease, the two most distinguishing features of systemic sclerosis are: o Striking cutaneous changes Notable skin thickening. This is the most easily recognized manifestation of scleroderma. Raynaud's phenomenon This is the first manifestation of disease in almost every patients. Dysphagia Attributable to esophageal fibrosis and its resultant hypomotlity is present in more than 50% of patients. Remember, Whenever skin thickening is present along with Raynaud's phenomenon, it is almost always a case of scleroderma". These two features are not present in any other multisystem disease whose clinical features overlap with that of systemic sclerosis e.g. SLE, rheumatoid ahritis, inflammatory myopathy, Sjogren syndrome". Although skin changes and Raynaud's phenomenon are the major diagnostic clues, scleroderma is a multisystem disease that most commonly targets peripheral circulation, muscles, joints, gastrointestinal tract, lung, hea and kidney. So, the symptoms encountered in early presentation of scleroderma include musculoskeletal discomfo, fatigue, weight loss, and hea burn and dysphagia associated with gastroesophageal reflex disease (GERD). When these symptoms are accompanied by the skin thickness and Raynaud c phenomenon, diagnosis ofscleroderma should be considered. Role of autoantibodies in the diagnosis of scleroderma Autoantibodies are found in nearly every patient with scleroderma (sensitivity >95%), but they are not specific for scleroderma0. Scleroderma is associated with wide array of autoantibodies. Two ANA'S which are more or less unique to scleroderma are: Antitopoisomerase antibody (20-40%) Seen in patients with diffuse systemic sclerosis Patients with this autoantibody are more likely to havepulmonary .fibrosis and peripheral vascular disease Patients with these autoantibodies have poor prognosis Anticentromere antibody (20-40%) These autoantibodies are seen in patients with limited systemic sclerosis