Systemic sclerosis, or scleroderma, is a connective tissue disease characterised by vascular changes that induce fibrosis in the skin and other organs and systems

In its advanced form, it can lead to impairment of the musculoskeletal, skin and respiratory systems and, for this reason, requires appropriate, multidisciplinary rehabilitation therapy.

Alterations of the hands and face: a disabling symptom of systemic sclerosis

Characteristic of systemic sclerosis is the impairment of the skin, a very debilitating aspect present in all patients and with a centripetal trend, which affects early and electively the hands and the face, but can later extend to other skin sites.

Skin involvement can be divided into three successive phases, which are particularly evident when talking about the hands: the oedematous phase, in which the movement of the fingers and the function of the hands is restricted; the sclerotic phase, in which fibrosis of the skin occurs, increasing in consistency and becoming inelastic, shiny and adherent to the subcutis, which, in turn, by shrinking, determines the deformities of the hands and face; and the atrophic phase, in which the skin thins, the fingers stiffen in flexion until the deformity called “claw hands”, and the movements of the hands and wrists are further reduced. Scleroderma alterations, therefore, are significantly disabling for the patient, as they lead to impaired use of the limbs and painful ulcerations.

The anatomical and functional alterations of the hands and face are also caused and aggravated by impairment of the musculoskeletal system and microcirculation.

The face, for example, shows radial folds around the mouth, a sharp nose and flattening of the wrinkles.

But the consequences of scleroderma can also be seen at the temporomandibular level, with pain and reduced movement of the jaw, dental and paradental changes and cleft palate.

Rehabilitation therapy for hands and face

In systemic sclerosis, rehabilitation treatment is effective in preventing and reducing impairment of the hands and face, in contrast to drug therapy, which has little effect.

However, in order to ensure recovery of joint mobility and skin elasticity and to limit deformities, early diagnosis and early action are essential.

As far as the hands are concerned, the rehabilitation pathway is aimed at improving limb movement and strength and involves a programme of stretching exercises, manipulations, mobilisation of the fingers and massages.

The first rehabilitation approach should focus on the skin.

The possible use of braces can be helpful in protecting and resting the joints, but careful evaluation by the doctor is essential not only before applying the brace, but also throughout its use.

In facial rehabilitation, on the other hand, the doctor’s goals are to improve oral functions, such as chewing and swallowing, recover facial expression and restore correct head alignment.

Facial stretching exercises involve exaggeration of normal facial movements and muscle strengthening exercises in the mouth. In addition, a series of manual techniques and manipulations are to be added.

The face is an intimate and delicate region of the body, and rehabilitation work for patients can be particularly tiring.

Comprehensive rehabilitation: a necessity for improving the quality of life of systemic sclerosis sufferers

In general, scleroderma debilitates the patient globally, causing significant musculoskeletal changes, starting with the cervical spine.

This requires a rehabilitation programme that takes this into account and is aimed at improving posture, breathing, muscle tone and the general state of health.

What kind of therapy are we talking about? Aerobic exercises of increasing intensity in the absence of pulmonary involvement, for example, combined with muscle strengthening, breathing exercises and stretching.

But also therapies that improve blood circulation through the production of heat, e.g. using heated pools, thermal mud and hot water packs.

Systemic sclerosis is a complex disease, which requires continuous and constant rehabilitation treatment and therefore requires the involvement and participation of the patient to increase the chances of successful treatment.

This can lead to general fatigue and a depressed mood, especially because of the constant therapeutic commitments. For this reason, the doctor will take care not to tire the patient excessively and will propose targeted and necessary therapeutic interventions.

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Source:

Humanitas