Scleroderma is a multisystem disease characterized by a relatively
common inflammatory changes, and sclerotic vascular diseases affecting
the skin and internal organs, especially lungs, heart and
gastrointestinal tract. The mean age of disease onset is 30-50 years
with a female predominance of 4:1.
Two forms of the disease are recognized:
* Systemic Scleroderma located (60%) in women and generally
occurs in people older than those who diffuse systemic scleroderma. In
this case the skin damage is limited to hands, feet, face and forearms,
and organ damage occurs late;
* Sclerodemia systemic spread. Onset is quick and is
characterized by extensive involvement of the trunk early systemic and
determination.
A symptom is present in all people with Raynaud’s phenomenon,
characterized by ischemia in the fingers to cold exposure, which may
precede the disease for years. Also, people complain of pain and reduced
mobility in the fingers and knees and may have arthritis.
Skin Damage
1. Effect on the hands and feet. Initial: Raynaud’s phenomenon occurs
(hands become pale when exposed to cold, and cyanotic), sclerosis
(thickening and reduced skin elasticity), finger pulp sores that heal
with scars;
2. Impaired face
* Initial: periorbital edema occurs;
* Late: skin edema and fibrosis leading to the appearance of a
mask layout, with the disappearance of wrinkles, thinning lips, oral
aperture reduction, narrowing the nose, so that eventually the patient
looks younger than it really is.
Organ damage
* Esophageal Damage: dysphagia occurs (the patient swallows hard) initially solid and then liquid;
* Effect on Gastrointestinal: constipation, diarrhea, bloating, malabsorption (decreased absorption of nutrients);
* Impairment of pulmonary alveoli, pulmonary fibrosis and inflammation, decreased oxygenation of the lungs;
* Impaired Cardiac conduction disturbances, heart failure, pericarditis;
* Renal impairment: occurs in approximately 45% of cases, can lead to kidney failure and malignant hypertension;
* Impaired musculoskeletal disorders: muscle weakness, carpal tunnel syndrome.
Treatment Options
The corticosteroids can be useful early in the disease. Other drugs
(Capro amino acid, D-penicillamine, colchicine) have not proven
effective. Immunosuppressive drugs (cyclosporine, methotrexate,
cyclophosphamide, mycophenolate mofetil) have improved skin symptoms,
but had a limited benefit on organ damage.
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