Scleroderma: definition, causes, symptoms, diagnosis and treatment

By Cristiano Antonino Last updated Jun 16, 2023

Scleroderma sees its peak onset in patients between the ages of 45 and 65 and, as is often the case with autoimmune diseases, it is women who are more affected, with a clear disproportion of 3-5:1

This is because, in general, the cells of the female immune system behave differently from those of the male immune system.

Indeed, women are genetically more active in immune responses, both humoral and cellular.

This is undoubtedly a double-edged sword because, while on the one hand it makes her more resistant to infections from outside, on the other hand it makes her more prone to developing autoimmune diseases, i.e. conditions characterised by an altered immune response towards proteins, tissues or even organs belonging to her own body, which suffer damage that will lead to pathological dysfunction.

Scleroderma: what is it?

Scleroderma – in Greek, literally ‘hard skin’ – is a chronic autoimmune disease affecting the skin.

An autoimmune disease occurs when the immune system, diseased, attacks its own tissues.

Scleroderma, also known as systemic sclerosis, occurs due to an exaggerated fibrosis (thickening) of tissues and internal organs, as well as an alteration of small-calibre vessels and abnormalities in the immune system.

The causes are unfortunately still unknown, but it has been hypothesised that environmental factors – such as exposure to solvents or toxins – may play a role in the onset of scleroderma.

Scleroderma: the symptoms and signs by which it manifests itself

Scleroderma (or systemic sclerosis) is a connective tissue disease involving the vascular system, the skin.

The most characteristic sign with which scleroderma manifests itself is skin thickening, usually localised at the hands, which eventually present an increasingly severe and progressive deformity that progresses from these to the wrist, the arm, and on to the face.

Depending on the severity and extent of the skin thickening, various forms of scleroderma can be distinguished:

limited scleroderma – skin sclerosis extending proximally to the elbows or knees or affecting the trunk or abdomen;
diffuse scleroderma – cutaneous sclerosis distal to the elbows and knees; also includes the CREST form (cutaneous calcinosis, Raynaud’s, oesophagopathy, sclerodactyly, telangiectasias)
sine scleroderma form with absence of cutaneous sclerosis, but presence of typical involvement of internal organs and disease-specific antibodies.

Scleroderma: diagnosis

The earliest clinical manifestations are borne by the microvascular and autonomic nervous system and consist of Raynaud’s phenomenon – a phenomenon with which scleroderma can begin to manifest itself – which consists of a sudden change in the colour of the extremities that first become pale, cyanotic and then dark red due to reduced blood flow.

Raynaud’s phenomenon is of vasomotor origin and is a sign that may also imply other diseases, so it is not only prognostic of scleroderma.

Other tests, such as the presence of anti-Scl70 or anticentromere antibodies, ANA and anti-ENA autoantibodies, capillaroscopy and other typical patterns of the disease, are required for a diagnosis of scleroderma.

Scleroderma: the most indicated therapies

Unfortunately, there are no drugs that can cure scleroderma.

Therefore, the most indicated therapies in the event of a diagnosis only serve to contain the most obvious symptoms of the disease and delay its advancement and the possible onset of complications as much as possible.

The most successful drugs to date are vasodilators, which improve blood circulation and reduce pressure; antacids for the stomach; antiarrhythmics for heart failure; and immunosuppressants to treat the musculoskeletal system.

In order not to further slow blood circulation, patients diagnosed with scleroderma are advised to keep the temperature of their living quarters above 20°C.

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