Pathology definition - Scleroderma
Scleroderma
Scleroderma is also known as progressive systemic sclerosis. It may present as diffuse scleroderma or CREST syndrome. Diffuse scleroderma may present with anti - Scl 70 antibody while CREST syndrome may present with anti centromere antibody.
Diffuse scleroderma is presented with symptoms and signs such as abdominal pain, dysphagia, shortness of breath (pericarditis, myocardial fibrosis, pulmonary hypertension and interstitial fibrosis), arrhythmias, malabsorption syndrome (due to atrophy and fibrosis of the muscularis with loss of microvilli and villi of the small bowel), tendon friction rubs ( hypertrophy of the synovial tissue and inflammation of the synovium) over the knee, ankles and wrist as well as thickening of the skin with increase and decrease pigmentation of the skin.
CREST syndrome mostly affect the face and the hands.CREST syndrome may include calcinosis ( subcutaneous calcification due to dermal fibrosis as a result of edema and perivascular lymphocytic infiltration), Raynaud phenomenon, dysmotility of the esophagus, sclerodactyly or claw like hands and telangiectasia.
Scleroderma is associated with dysregulation of the immune system. There will deposition of the collagen and fibrosis of the skin, lung, heart, esophagus, kidney and joints. Scleroderma mostly affect middle and elderly female.
Scleroderma may affect the kidney with vascular fibrinoid necrosis and collagenous deposition in the vessel wall with thickening of the intimal walls.
Supportive and symptomatic treatment are considered in patient with scleroderma.
Scleroderma is also known as progressive systemic sclerosis. It may present as diffuse scleroderma or CREST syndrome. Diffuse scleroderma may present with anti - Scl 70 antibody while CREST syndrome may present with anti centromere antibody.
Diffuse scleroderma is presented with symptoms and signs such as abdominal pain, dysphagia, shortness of breath (pericarditis, myocardial fibrosis, pulmonary hypertension and interstitial fibrosis), arrhythmias, malabsorption syndrome (due to atrophy and fibrosis of the muscularis with loss of microvilli and villi of the small bowel), tendon friction rubs ( hypertrophy of the synovial tissue and inflammation of the synovium) over the knee, ankles and wrist as well as thickening of the skin with increase and decrease pigmentation of the skin.
CREST syndrome mostly affect the face and the hands.CREST syndrome may include calcinosis ( subcutaneous calcification due to dermal fibrosis as a result of edema and perivascular lymphocytic infiltration), Raynaud phenomenon, dysmotility of the esophagus, sclerodactyly or claw like hands and telangiectasia.
Scleroderma is associated with dysregulation of the immune system. There will deposition of the collagen and fibrosis of the skin, lung, heart, esophagus, kidney and joints. Scleroderma mostly affect middle and elderly female.
Scleroderma may affect the kidney with vascular fibrinoid necrosis and collagenous deposition in the vessel wall with thickening of the intimal walls.
Supportive and symptomatic treatment are considered in patient with scleroderma.
