RESUMO
Systemic sclerosis (SSc) is a connective tissue disorder of unknown aetiology. A small subset (10%) of patients with limited systemic sclerosis have all other features of the disease without any skin involvement and is known as systemic sclerosis sine scleroderma (ssSSc). Severe Critical Limb Ischaemia is rare in sine scleroderma.The present case showed severe critical limb ischaemia with severe PAH,Esophageal dysmotility,Glomerulonephritis(a rare association) with hypertension. Although skin thickening is considered as a hallmark of systemic sclerosis, there should be a high index of clinical suspicion in patients presenting with possible manifestations of systemic sclerosis without sclerodermatous cutaneous involvement because early diagnosis and treatment can reduce the morbidity and mortality in it.
