Scleroderma (or systemic sclerosis) is a disease characterized by abnormalities in the functioning of small blood vessels and the immune system, ultimately leading to inflammation and excessive fibrosis of the skin an...Scleroderma (or systemic sclerosis) is a disease characterized by abnormalities in the functioning of small blood vessels and the immune system, ultimately leading to inflammation and excessive fibrosis of the skin and various organs, including the heart. Management must be multidisciplinary, to avoid complications that are often serious. We report the case of a 20-year-old patient with no known cardiovascular history who consults for dyspnea, and retrosternal pain associated with a dry cough. On physical examination, she had tachycardia, swelling of the lower limbs, jugular turgidity, and deafening heart sounds. Cardiac Doppler ultrasound shows dilation of the right cavities, paradoxical septum and significant pulmonary arterial hypertension, pericardial effusion of medium abundance. On oral examination, it presents an ulceration of the lips, dermatological examination finds scattered hypo chromic spots in the body, more accentuated in the face. Before the hypo chromic dermatosis, a dermatological consultation was carried out with an autoimmune assessment that came back positive for systemic scleroderma.展开更多
文摘Scleroderma (or systemic sclerosis) is a disease characterized by abnormalities in the functioning of small blood vessels and the immune system, ultimately leading to inflammation and excessive fibrosis of the skin and various organs, including the heart. Management must be multidisciplinary, to avoid complications that are often serious. We report the case of a 20-year-old patient with no known cardiovascular history who consults for dyspnea, and retrosternal pain associated with a dry cough. On physical examination, she had tachycardia, swelling of the lower limbs, jugular turgidity, and deafening heart sounds. Cardiac Doppler ultrasound shows dilation of the right cavities, paradoxical septum and significant pulmonary arterial hypertension, pericardial effusion of medium abundance. On oral examination, it presents an ulceration of the lips, dermatological examination finds scattered hypo chromic spots in the body, more accentuated in the face. Before the hypo chromic dermatosis, a dermatological consultation was carried out with an autoimmune assessment that came back positive for systemic scleroderma.
