Skin involvement

The term ‘Scleroderma’ literally means ‘hard skin’ in Greek, this describes how affected skin feels to touch, indeed some patients describe their skin as feeling ‘woody’, and it is this finding which allows your doctor to make the diagnosis of Scleroderma. The affected skin may feel tight and thickened so that it is difficult to pinch, this is due to swelling and excessive deposition of fibrous tissues, the skin may appear shiny having lost hair and wrinkles. The skin can be affected in any area of the body but in the form of scleroderma termed ‘systemic sclerosis’, which can also affect the internal organs, the skin tends to change initially on the fingers and hands. This can then restrict the movement of joints e.g. making it difficult to straighten fingers or make a fist. In approximately one third of patients the skin tightening may then spread further up the arms above the elbows and sometimes onto the trunk (the thighs can also be affected). This type of scleroderma is termed diffuse systemic sclerosis.

As recently as 1990 it had been generally thought that the extent of affected skin got progressively worse but doctors treating larger numbers of patients with severely affected skin disease noted that this was often not the case. The Royal Free Hospital is a major centre in the UK for assessment and treatment of systemic sclerosis and we have seen many patients with the diffuse subtype of this condition. We thought we could study this further. So with funding from the Raynaud’s and Scleroderma Association we have undertaken a study to assess whether the extent and severity of skin tightening can change over time in a group of 192 patients, who had all attended the Royal Free Hospital Scleroderma Clinic. These patients were all seen at the Royal Free Hospital from within one year of the illness becoming noticeable and had attended the clinic in the period since 1985. As standard practice these patients had come to the clinic at least once a year for an assessment that included a measurement of skin tightening. This was done by the doctor pinching skin in a number of different body surface areas and giving a numerical score for each area from 0 to 3 depending on how severely the skin was tightened. The total numerical score is termed the modified Rodnan skin score.

What this study has shown is that in the majority of patients the tightened skin affected by systemic sclerosis can soften and often does so only a few years after it became tight. This knowledge allows us to advise those patients with severely affected skin who have recently developed diffuse systemic sclerosis that there is a high probability that some of the skin will soften within a few years. The treatments that we give patients early on in the condition aim to prevent skin becoming tighter and to help the skin already affected to soften more effectively and quickly, and of course to reduce the risk of complications affecting internal organs e.g. lungs. Although this study confirms that skin can soften it does not tell how this happens that it why we rely on the help of patients in providing us with samples of skin and blood which is essential for our scientists to continue to carry out research into this complicated condition.

We gratefully acknowledge funding from the Raynaud’s & Scleroderma Association to support this research.

Dr Lynne Shand, - Clinical Research Fellow, Scleroderma Clinic, Rheumatology Dept, Royal Free Hospital