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Localised Scleroderma -
MORPHEA
Taken from a publication by the
Raynaud's & Scleroderma Association of the U.K.
Unlike systemic sclerosis, localised scleroderma
seldom affects the internal organs of the body. There are two types of
localized scleroderma -
morphea and linear. One of the important differences between morphea and
linear is that linear scleroderma tends to involve not only the skin but
also the deeper tissues with fixation to
muscle and bone.
Morphea is the name given to localised patches of hardening (sclerosis)
which can affect the skin. So why is it not called scleroderma, which
means hardening of the skin? Probably because
it helps to have a different name to distinguish morphea from the more
generalized sclerosis, which affects both the skin and other organs in
systemic sclerosis. Although there is some
overlap, morphea and systemic sclerosis
are better regarded as separate entities.
Morphea usually appears spontaneously, as asymptomatic hard patches in
the skin. These initially have a faint purplish colour, fading centrally
to develop a waxy, ivory appearance
with purple coloured edges. The patches are round or oval and may be
multiple, affecting almost any part of the body. Sometimes the lesions
are deeper , involving tissue beneath the skin. Only rarely does morphea
become
generalised.
Early morphea has an inflammatory stage where the skin
often itches. This is followed by one or more slowly enlarging patches
or plaques. The plaques vary in size, they are often oval in shape,
occur most commonly on
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the trunk but may also involve the extremities and
rarely the face.
Morphea is three times more common in women than men. It most frequently
occurs between the ages of 20 -40 years but 15% of cases occur in
children. Most lesions of morphea improve with time -typically three to
five years-and eventually clear almost completely but residual
pigmentation may persist. Generally treatment is not
indicated, although local steroid applications may be used to hasten
clearance. ..
Treatment varies according to the extent of the localized scleroderma,
the areas involved and the age of the patient.
Not every patient with morphea will require treatment. Therapy, however,
is indicated for patients whose lesions are increasing in number of
where there is growth impairment or functional incapacity .
The aim of the drug treatment is to stop the ongoing
inflammation which causes these skin lesions. Drug
treatment .aims to stop the skin lesions progressing so fast and to
soften the skin a little, but at present it cannot
completely remove the lesions. Currently a number of drugs are being
used and new treatments are always being examined.
In the management of local disease, it is important for all lesions to -
be carefully noted and also at times to
measure the size of a skin lesion as well ,as the length of the affected
limb and the opposite limb. Monitoring in this way is very important to
assess the state of the illness and its response to treatment. For just
one or two small patches of morphea all that will be needed will be a
local
cream. If it becomes more generalised, other treatment may need to be
considered, but this is rare.
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