Scleroderma is a systemic disease of connective tissue, the essence of which is a progressive fibrosis of the skin and internal organs. It comes in two forms - generalised and diffuse. In terms of skin lesions, the most common lesions that develop are oedematous or atrophic, usually in the hands and face.
A characteristic feature of generalised scleroderma is the presence of Raynaund's sign, which may appear even several years before the onset of scleroderma symptoms.
Scleroderma is a systemic disease of connective tissue. In the generalised form, lesions involve the skin, vascular system, skeletal and muscular systems and internal organs. In the limited form, however, the lesions affect only the skin or the skin and subcutaneous tissue.
A characteristic feature of generalised scleroderma is Raynaund's sign, which is a paroxysmal blurring of the fingers (less commonly the feet) due to arterial spasm with subsequent bruising and congestion, often accompanied by pain. Raynund's sign can appear even several years before the onset of scleroderma symptoms.
With regard to skin lesions, oedematous or atrophic lesions usually develop in the upper limbs and face. In the face, atrophic changes of the nose and redness of the lip with radial furrowing in its area are characteristic. In the hands, there is sclerodactyly and thinning of the skin on the fingertips with necrotic lesions. In advanced cases, contracture and immobilisation occur.
The organ changes that develop in the course of scleroderma most commonly involve:
- thecardiovascular system - cardiac arrhythmias and pericarditis may develop,
- lungs - in the form of fibrosis symptoms, pleuritis,
- oesophagus - atony of the lower part of the oesophagus, causing swallowing disorders,
- kidney - changes of the malignant hypertension type,
- skeletal system - inflammation of tendon capsules accompanied by joint pain, accelerated development of osteoporosis,
- muscular system - hypertrophy of intermuscular connective tissue.
Treatment is directed against the individual components of the disease, i.e. inflammation, vascular changes and the fibrotic process. Causal treatment is not possible, due to a pathogenetic mechanism that is not fully understood.