Q and A

Your questions answered by our medical professional Prof Chris Denton.

I think my daughter may have Raynaud's as her little toe keeps going completely white - how can my GP diagnose childhood Raynaud's?
It is difficult to diagnose Raynaud's in children, whose extremities are often much colder and prone to colour change, compared with adults.Although Raynaud's can occur in young people, it is usually identified by very dramatic blue or red discoloration and complaints of pain or obvious problems with gripping objects or using hands. Even when diagnosed, any medical treatment for Raynaud's would usually be delayed until teenage years when Raynaud's is more common and more drugs could be considered.

My 15 yr old son has had Morphea for about 6 years and in the last 18 months his wrist has become completely 'stuck' in ulnar drift position. I want to know what treatment is available. Also the doctors have said that the scleroderma may be affecting internal organs - what tests should I be asking for?
Morphoea describes all forms of localised scleroderma that affect the skin and underlying tissues but do not generally have any effect on internal organs such as the lungs, heart or blood vessels. Very occasionally a patient with systemic sclerosis also develops skin morphoea but this is a coincidence of two diseases not progression between the conditions. Morphoea is the commonest form of scleroderma to develop in children. To exclude internal organ disease in any patient it is usual to perform standard heart, lung and kidney tests. In typical morphoea these are expected to be normal. The effect of morphoea on bone and joint growth at the sites of skin change can lead to deformity as you describe, with bending of the wrist outwards (“ulnar") and may prompt a change in medical treatment. Physiotherapy and exercises are also important especially if the limbs are still growing.



I have had a 6 month course of cyclophosphamide given every four weeks intravenously. I am currently feeling really good and wanted to know whether this will last?
Cyclophosphamide is a chemotherapy drug that reduces immune system overactivity and has been shown to benefit skin and lung fibrosis in scleroderma. Although it can have side effects these are much less when given as a monthly intravenous treatment than tablets every day. Usually treatment is followed by tablet therapy with another immune suppressive such as mycophenolate mofetil for at least 18 months (often longer) to maintain the benefit that can wear off otherwise.

I have been diagnosed with scleroderma, do I need to stay out of the sun?
There is no evidence that scleroderma is aggravated by sun exposure, unlike related diseases such as lupus (SLE) or dermatomyositis, where sun exposure can definitely worsen skin rashes and may aggravate internal organ disease. However, it is still sensible to use high factor sunblock and protect the skin. If you are taking immunosuppression such as mycophenolate mofetil it is advised to avoid sun exposure as there can be increased risk of sun induced skin cancers, although this is rare. Diffuse scleroderma is often associated with increased pigment in the skin, so patients can become generally much darker, but this is not necessarily associated with worsening skin fibrosis.

I have received a letter from my GP advising me to have influenza vaccination due to my age (65). I have Raynaud's and scleroderma, will this vaccine affect any of my medication or lead to side effects due to my conditions?
In general if you have any chronic disease that could affect the lungs or are taking medication that reduces immune activity you should definitely have the influenza vaccination, as you will be more prone to this illness and to its complications. There is no evidence that vaccination worsens established scleroderma.

I have Lichen Sclerosus (LS) and I've often wondered how the “sclerosus" aspect of LS relates to scleroderma?
Lichen sclerosus is a distinct skin disease from scleroderma with a somewhat similar name. It causes inflammation, itching and thickening in some area but later the affected skin can becomes very thin. When a biopsy (sample) of skin is examined under the microscope the appearance of lichen sclerosus is very different from morphoea but occasionally the conditions occur together in the same patient.Sclerosis means “hard" and the skin can be hard or locally thicken in scleroderma and in lichen sclerosus, hence the similar names.

What affect does Raynaud's have on blood tests?
Raynaud's that is not associated with any underlying disease, termed primary Raynaud's is not associated with any blood test abnormality and so blood tests can be important in making the diagnosis. In secondary Raynauds there can be associated connective tissue or rheumatic diseases and blood tests such as antinuclear antibody (ANA) may be found. These tests are useful in diagnosing cases of secondary Raynaud's. They can also predict cases of Raynaud's that are at increased risk of progressing to a defined disease such as scleroderma or lupus in the future. Together with microscopic examination of the small blood vessels around the finger nails (capillaroscopy) these ANA blood tests are very sensitive and have strong predictive value if positive and are even more reassuring that no connective tissue disease progression will occur when they are negative.

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