Systemic sclerosis sine scleroderma is a rare form of limited systemic sclerosis, but without skin involvement. It does not differ in its clinical or laboratory features and prognosis from classical systemic sclerosis. In the absence of cutaneous signs/symptoms, its diagnosis is delayed leading to significant morbidity and mortality. We report the case of a 28-year-old male who presented with dyspnoea on exertion and Raynaud's phenomenon. Diagnostic evaluation confirmed systemic sclerosis sine scleroderma and pulmonary artery hypertension.
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