Scleroderma
The word "scleroderma" literally means "hard skin", and it is used for two very different things.
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Medicines commonly prescribed for Scleroderma
For informational purposes only. Always consult a doctor before using any medicine.
Dosage form: INJECTABLE, 17.5 mgActive substance: methotrexateManufacturer: Medac Gesellschaft Für Klinische Spezialpräparate GmbhPrescription requiredDosage form: TABLET, 2.5 mgActive substance: methotrexateManufacturer: Orion CorporationPrescription requiredDosage form: INJECTABLE, 50 mg/mlActive substance: methotrexateManufacturer: Medac Gesellschaft Für Klinische Spezialpräparate GmbhPrescription required
The word "scleroderma" literally means "hard skin", and it is used for two very different things. One is a skin condition, commonest in children, that stays as patches and bands of thickening and never reaches the internal organs. The other is systemic sclerosis, in which not only the skin hardens but also the lungs, the gullet, the blood vessels and the kidneys. The two are constantly confused, and the confusion is costly: someone with a single patch on the shoulder reads about lung damage and takes fright over nothing, while someone who really does have systemic sclerosis spends years not knowing which checks they are owed. So we start with the distinction — here it matters more than anything else.
Two conditions under one word
Localised scleroderma, which doctors call morphoea, is a condition of the skin and the tissue immediately beneath it. Areas of thickening appear: a patch or a band where the skin becomes firm and shiny, changes colour and loses its hair. Nothing else happens. There is no Raynaud's phenomenon, the internal organs are not affected, and it does not lead to life-threatening complications.
Systemic sclerosis is a condition of the blood vessels and connective tissue throughout the body. The skin thickens here too, but the important part happens inside: in the lungs, the gullet, the heart and the kidneys.
And the point this section exists for: localised scleroderma does not turn into systemic sclerosis. They are two different conditions, not two stages of one. If a doctor has said "morphoea" or "localised form", everything you read below about lungs, blood pressure and kidneys does not apply to you.
A rheumatologist tells them apart on quite specific grounds: whether the fingers go white in the cold, whether the fingers are puffy and thickened, what the capillaries at the base of the nail look like under magnification, and which antibodies are in the blood. If doubt remains, breathing tests and an echocardiogram are added to settle the question.
Localised scleroderma
It occurs in both children and adults, more often in children, and it looks different from case to case:
- plaque morphoea — oval patches on the trunk, from a few centimetres upwards. At first they have a lilac rim and look slightly swollen; later they become firm and smooth and either lighten or, on the contrary, darken. Commoner in adults;
- linear morphoea — a band of thickening running down an arm or a leg, sometimes on the forehead and scalp. This is mainly the childhood variant and it needs closer attention: the band can reach the muscle and bone beneath it and, if it crosses a joint or a growth plate, the limb grows less than the other one. A band on the forehead and scalp sometimes involves the eye, so these children are also seen by an ophthalmologist.
The usual course is this: the patch stays active for a few years, then stops spreading and remains as a thickened light or dark mark. The mark may not disappear, but the disease itself has finished.
Treatment depends on how active and how deep it is. Small, quiet patches need no more than treatments applied to the skin and good moisturising. Active, spreading patches, and any linear form in a child, need something more substantial: usually medicines that damp down the inflammation from within, given over a long course, plus monitoring to catch the point where it stops. Exercises and stretching are added if the thickening pulls on a joint.
Systemic sclerosis: how it starts
It affects mostly women and usually begins somewhere between thirty and fifty.
The first sign is almost always Raynaud's phenomenon, and it appears years before anything else. In the cold or with stress the fingers go suddenly white, then blue, and on warming up they turn red, tingle and hurt. Raynaud's on its own is very common and generally means nothing. But there are pointers that call for investigation: it began after the age of thirty, it affects the fingers unevenly, painful small ulcers or pitted scars appear on the fingertips, or the fingers are puffy in the mornings. That is a reason to see a rheumatologist, not to buy thicker gloves.
Then the skin joins in. It starts not with hardness but with swelling: rings become tight and the fingers look puffy. Later the skin on the fingers thickens, it becomes hard to pinch into a fold, and the fingers straighten less well. On the face the mouth opening narrows and fine thread veins appear.
Two variants are distinguished by how far the skin thickening goes, and the difference is a practical one:
- the limited form — the skin is affected on the hands, forearms, feet, lower legs and face. It develops slowly, over many years, and usually starts with a long period of Raynaud's. The main threat here is a delayed one: raised pressure in the lung vessels years later;
- the diffuse form — the thickening spreads quickly to the upper arms, trunk and abdomen. There is often weight loss, weakness, and joint pain and stiffness. It is precisely in the early years of this form that the risk of lung involvement and of renal crisis is highest, which is why monitoring at that stage is closest.
What happens to the internal organs
Gullet and bowel. Affected in most people, and among the earliest things to go. Acid comes back up into the gullet, bringing heartburn, a lump behind the breastbone, difficulty swallowing dry food and a night-time cough. Later, feeling full early, bloating and alternating diarrhoea and constipation may be added. This is not a trivial matter: continuous reflux damages the gullet and makes the lung situation worse, so it is treated seriously and continuously.
Lungs. These are the leading cause of death in systemic sclerosis, and most of the monitoring is aimed at them. Two separate problems are possible. The first is scarring of the lung tissue, which brings increasing breathlessness on exertion and a dry cough. The second is pulmonary hypertension, raised pressure in the blood vessels of the lungs, which shows itself as breathlessness, tiring quickly, and sometimes dizziness or fainting on exertion. What makes them treacherous is that both creep up unnoticed: a person simply stops climbing stairs as easily as before and puts it down to age or weight.
Heart. Rhythm disturbances, a sense of missed beats and fluid collecting around the heart are all possible.
Kidneys. For a long time they give no sign at all, and then they can produce a sudden and dangerous complication — the subject of the next section.
Fingers. Poor blood flow leads to painful ulcers on the fingertips that heal badly. They must not be left: they infect easily and in severe cases go on to tissue death. An ulcer that appears is a reason to see a doctor, not to apply home remedies.
Monitoring: what is checked and how often
It is worth understanding the logic. In systemic sclerosis the lungs and heart are not examined when symptoms appear but to a timetable, regardless of how well you feel. The reason is simple: breathlessness appears once an appreciable part of the function has already been lost, and by then it is too late to get it back. Finding it early changes the outcome.
The usual set is this:
- breathing tests — spirometry together with a measurement of the lungs' transfer capacity. Done at diagnosis and then regularly, usually once a year, and more often in the early years of the diffuse form;
- an echocardiogram — likewise yearly: it is the first thing to notice rising pressure in the lung vessels;
- a CT scan of the chest — at diagnosis and afterwards as indicated;
- blood tests of kidney function, an ECG, and examination of the capillaries at the nail fold.
And one thing people are often not told. In systemic sclerosis blood pressure is measured at home, by yourself and regularly, particularly in the first years of the illness and particularly in the diffuse form. A blood pressure monitor here is not a luxury but part of the treatment. Get into the habit of writing the readings down: a doctor needs not a single figure taken in the clinic but your usual level, so that a spike can be recognised against it.
Renal crisis: what must not be blamed on stress
Scleroderma renal crisis is the most dangerous complication of this disease and one of the few situations here where it is a matter of hours. The small vessels of the kidney narrow abruptly, blood pressure shoots up and the kidneys start failing within days. This complication once almost always ended in loss of the kidneys; today, if treatment starts immediately, the outcome is quite different. Everything depends on speed.
Seek emergency help immediately if you have systemic sclerosis and you develop:
- a sharp rise in blood pressure, especially if it was normal before, or readings clearly above your usual level;
- a new severe headache, often at the back of the head;
- blurred vision, floaters or flashes in front of the eyes;
- breathlessness you did not have before, and swelling;
- a sudden fall in the amount of urine you pass;
- seizures, confusion or unusual drowsiness.
This calls for an ambulance — in European countries on the single number 112. And the whole point of this section: a jump in blood pressure in someone with systemic sclerosis must not be put down to stress, a bad night, the weather or coffee. That is the commonest reason time gets lost. Tell the doctor that you have systemic sclerosis: how quickly the right treatment is started depends on that sentence.
One specific risk is also worth knowing: high doses of steroid medicines raise the likelihood of a renal crisis. That is why in this disease steroids are prescribed cautiously, in short courses and at the lowest dose, and never taken "for the joints" on your own initiative.
Treatment and everyday life
There is as yet no medicine that switches the whole disease off. But there is treatment organ by organ, and it genuinely changes both how you feel and how long you live. A rheumatologist handles it together with a respiratory physician, a cardiologist and a gastroenterologist.
- Vessels and Raynaud's. Medicines that widen the blood vessels reduce how often and how severely attacks occur and help finger ulcers heal; for stubborn ulcers there are stronger regimens, including intravenous ones.
- Lungs. For scarring of the lung tissue, medicines that damp down immune inflammation are used, together with drugs that slow fibrosis. For pulmonary hypertension there are specific treatments that appreciably prolong life. That is exactly why the scheduled checks are worth doing.
- Gullet. Acid-reducing medicines are usually taken continuously; small frequent meals, not eating for three hours before bed and raising the head of the bed all help.
- Skin and joints. Daily moisturising, gentle care, exercises and stretching are not cosmetic: they are how the movement of the hands and face is preserved.
What is up to you: keep the hands, feet and the whole body warm, and put gloves on in advance rather than once the fingers have already gone white; stop smoking, because tobacco narrows exactly these vessels and leads directly to finger ulcers; protect the fingers from cuts and burns; keep moving regularly. Pregnancy with systemic sclerosis is possible, but it is planned ahead and with a doctor: some medicines are incompatible with pregnancy, and the timing is chosen for a quiet phase of the disease.
Online consultation
The central question of this page is the easiest one to settle remotely: which of the two you actually have, a patch on the skin or a systemic disease. The doctor will go through the dermatologist's report, ask about the fingers in the cold, puffy hands, heartburn and breathlessness, and tell you whether the programme of checks you have just read about applies to you at all. If systemic sclerosis is already diagnosed, a remote appointment is a practical way to keep the monitoring on track: to check that all the due tests have been done and when the breathing tests and echocardiogram should be repeated, to go over your home blood pressure records, to discuss reflux and the care of skin and fingers, and to prepare for a conversation about pregnancy. The signs of renal crisis in the section above are not assessed remotely — with those, call an ambulance.
This material is for information only and does not replace medical advice.
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