En-coup-de sabre is a form of

Systemic sclerosis Systemic sclerosis, or scleroderma, is a generalised disorder of connective tissue affecting the skin, internal organs and vasculature. It is characterised by sclerodactyly in combination with Raynaud's and digital ischaemia It is subdivided into diffuse cutaneous systemic sclerosis (DCSS: 30% of cases) and limited cutaneous systemic sclerosis (LCSS: 70% of cases). Many patients with LCSS have features that are phenotypically grouped into the 'CREST' syndrome (Calcinosis, Raynaud's, oEsophageal involvement, Sclerodactyly and Telangiectasia). . Features that associate with a poor prognosis include older age, diffuse skin disease, proteinuria, high ESR, a low TLCO (gas transfer factor for carbon monoxide) and pulmonary hypeension. REF:DAVIDSON&;S PRINCIPLES AND PRACTICE OF MEDICINE 22nd EDITION PAGE NO 1112