Prognosis is generally poor, and the 5 year survival rate of patients with lymphangiosarcoma is less than 5%. Incidence
In the 1960s, the incidence five years after a radical mastectomy varied from 0.07% to 0.45%. Today, it occurs in 0.03% of patients surviving 10 or more years after radical mastectomy. History
It was discovered by Fred W. Stewart and Norman Treves in 1948. See also
Angiosarcoma
List of cutaneous conditions
Lymphangiosarcoma
Lymphedema
Postcardiotomy syndrome

References


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Scleroderma symptoms were first described in 1753 by Carlo Curzio and then well documented in 1842. The term is from the Greek skleros meaning "hard" and derma meaning "skin". Signs and symptoms
Potential signs and symptoms include:
Cardiovascular: Raynauds phenomenon (is the presenting symptom in 30% of affected persons, occurs in 95% of affected individuals at some time during their illness); healed pitting ulcers on the fingertips; skin and mucosal telangiectasis; palpitations, irregular heart rate and fainting due to conduction abnormalities, hypertension, and congestive heart failure
Digestive: gastroesophageal reflux disease, bloating, indigestion, loss of appetite, diarrhoea alternating with constipation, sicca syndrome and its complications, loosening of teeth, and hoarseness (due to acid reflux). Pulmonary: progressive worsening of shortness of breath, chest pain (due to pulmonary artery hypertension) and dry, persistent cough due to interstitial lung disease
Musculoskeletal: joint, muscle aches, loss of joint range of motion, carpal tunnel syndrome, and muscle weakness
Genitourinary: erectile dysfunction, dyspareunia, kidney problems, or kidney failure
Other: facial pain due to trigeminal neuralgia, hand paresthesias, headache, stroke, fatigue, calcinosis, and weight loss

Cause
Scleroderma is caused by genetic and environmental factors. Mutations in HLA genes seem to play a crucial role in the pathogenesis of some cases; likewise silica, aromatic and chlorinated solvents, ketones, trichloroethylene, welding fumes, and white spirits exposure seems to contribute to the condition in a small proportion of affected persons. Pathophysiology
It is characterised by increased synthesis of collagen (leading to the sclerosis), damage to small blood vessels, activation of T lymphocytes, and production of altered connective tissue.