ABOUT SCLERODERMA
Scleroderma
is an autoimmune connective tissue disease affecting blood vessels and collagen
production. It is more common in women than men. The cause is still largely
unknown and although there is no cure, there are many treatments to slow down
or halt disease progression. The word scleroderma, which means "hard skin",
is the generic term describing a group of related diseases in which the skin
becomes hardened and sclerotic. There are two kinds of scleroderma: systemic
sclerosis which affects the internal organs as well as the skin, and localised
scleroderma which affects a specific area of skin but not the organs.
LOCALISED SCLERODERMA
Localised scleroderma (not to be confused with limited) indicates that this
form of scleroderma is confined to the skin only and does not affect the internal
organs. It is more common in children. There are two main types, morphoea and
linear.
Morphoea
Patches of thickened skin, which may be rounded or oval in shape, can occur
all over the body. They are usually painless but can be itchy. The patches may
be lighter or darker than normal skin. Usually there are only a few patches
but sometimes the skin changes can be extensive, when the condition is known
as generalised morphoea.
Linear scleroderma
Thickened skin may occur in a linear pattern which can affect the head, leg
or arm. Unlike morphoea, linear scleroderma can affect not only the skin and
fatty tissue but also the underlying muscles and bone leading to growth deformities
in children. When linear scleroderma affects the face or scalp it is known as
"en coup de Sabre", as the scar resembles that caused by a knife or sword wound.
SYSTEMIC SCLEROSIS
The term systemic means that the scleroderma affects the internal organs. The
terms limited and diffuse refer to the extent of skin involvement.
Limited systemic sclerosis
(lcSSc) is also sometimes referred to as CREST syndrome, which stands for calcinosis,
Raynaud's Phenomenon, (o)esophageal dysfunction, sclerodactyly and telangiectasia.
Skin involvement is limited to the face, forearms, hands and lower legs, whereas
in diffuse disease the skin changes can affect the whole body. The severity
and problems created by skin changes will vary greatly between patients. Problems
include tightening of the skin around the fingers, the face and other areas
of the body causing contractures (fixed joints) and a small mouth (microstomia),
ulceration, dryness and irritation, broken blood vessels (telangiectasia) on
the face and hands and calcinosis protruding through the skin. In addition to
drug therapy, physiotherapy and exercises are important. Warm paraffin wax baths
can also be useful and moisturising the skin is essential.
The internal organs can be
affected in both limited and diffuse disease. Gastrointestinal (gut) involvement
affects most patients and investigations will determine which treatment will
be most effective.
Heart and lung involvement
can also be associated with both forms, although the heart is not as commonly
affected as the lung. Lung fibrosis is more common in diffuse patients and provided
it is diagnosed early enough, progression can be halted or slowed down.
A small percentage
(15%) of patients with lcSSc will develop pulmonary hypertension (PHT) a condition
affecting the vessels taking blood from the right side of the heart to the lungs.
These patients need to be seen by a cardiologist.
The kidneys are rarely
affected in limited disease, however approximately 5% - 10% of diffuse patients
will incur some form of renal involvement. It is very important for all patients
to have their blood pressure checked.
For a very small minority
the complications can be so severe that the disease is fatal. However the overwhelming
majority of people with Systemic Sclerosis can lead full and productive lives.
All patients with Scleroderma
should be closely followed up and have annual tests for organ problems.
For further information contact:-
The Scleroderma Society
3 Caple Rd
London NW10 8AB
Tel: 020 8961 4912
Email: info@sclerodermasociety.co.uk
www.sclerodermasociety.co.uk