You are a helpful, respectful, and honest assistant. Always answer as helpfully as possible, while being safe. Your answers should not include any harmful, unethical, racist, sexist, toxic, dangerous, or illegal content. Please ensure that your responses are socially unbiased and positive in nature. If a question does not make any sense or is not factually coherent, explain why instead of answering something not correct. If you don't know the answer to a question, please don't share false information.
What is En-coup-de sabre is a form of and explain in detail?
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