Scleroderma

The name of the disease comes from Greek and means “hard skin”. In scleroderma, cells called fibroblasts produce an excessive amount of collagen, the protein that gives connective tissue its strength. The collagen is deposited in the dermis and sometimes in the tissues beneath it, and the skin gradually loses its suppleness, becoming taut, shiny and hard to pinch between the fingers. The process is accompanied by early inflammation and by changes in the small blood vessels.

Who is affected. Localised scleroderma occurs at any age, but often begins in childhood or young adulthood and is more common in women. It is a rare condition and is not passed from one person to another. Genetic predisposition appears to play a part, yet most patients have no other family member with the same disease.

The localised forms. These account for the great majority of cases seen in dermatological practice:

• Plaque morphoea — one or more oval, indurated areas a few centimetres across, with an ivory-coloured centre and a violet halo at the edge, a sign of activity. It appears mainly on the trunk.
• Generalised morphoea — multiple plaques that merge and cover extensive areas of the trunk and limbs.
• Linear scleroderma — an indurated band following the axis of a limb; in children it may also involve subcutaneous tissue, muscle or bone, with a risk of asymmetric growth.
• The “en coup de sabre” form — a vertical band on the forehead and scalp, with a linear depression and sometimes hair loss along its path.
• Deep morphoea — involves the fascia and deeper tissues, with skin that appears bound down to the underlying planes.

The systemic form. Systemic sclerosis is a distinct disease in which skin thickening is accompanied by Raynaud phenomenon — fingers that turn white and blue in the cold — and by possible involvement of the oesophagus, lungs, kidneys or heart. It requires rheumatological assessment and follow-up. As a rule, the localised forms do not progress to the systemic form.

Causes and risk factors. The exact cause is not known. What is described is a combination of genetic predisposition, immune dysregulation with the production of autoantibodies, and damage to the small vessels, to which triggering factors may be added:

• local trauma, repeated injections or irradiation of a particular area;
• preceding infections;
• occupational exposure to organic solvents or silica dust, discussed mainly in relation to the systemic form;
• hormonal factors, the disease being more common in women.

What the patient notices and how it evolves. At first a slightly reddish or violet area may appear, sometimes tender, which hardens gradually over several months. The surface becomes smooth, shiny, free of hair and of sweating. The active, inflammatory phase usually lasts from a few months to a few years, after which the lesion stabilises and leaves an atrophic, hyper- or hypopigmented area. When a band of sclerosis crosses a joint, movement may become limited; in children, linear forms call for careful monitoring of limb growth.

What makes it worse. Cold, repeated trauma to the affected area, smoking, excessive dryness of the skin and prolonged immobilisation of neighbouring joints can accentuate the induration and the functional limitation. Maintaining mobility and keeping the skin moisturised are commonly recommended supportive measures.

At our practice, scleroderma is approached through the Kozak Method — internal and external treatment, individualised, based on consultation. Results differ from one patient to another.