What is scleroderma
An introduction to this complex and often challenging condition
When your child is diagnosed with scleroderma it can be extremely worrying, so it is important to have the right level of information and support. We are always here to provide any resources you may need and answer any questions, so please call our free helpline on 0800 311 2756.
There are different kinds of scleroderma, with varying degrees of severity. With scleroderma in children, it is the milder forms that are far more common than the more serious types.
Most children who have scleroderma are diagnosed with 'localised scleroderma'. This means that only the skin is involved; and the rest of the body will remain unaffected. There are several types of localised scleroderma that can occur in children:
Morphea is the most common form of scleroderma in children. It presents in irregular patches on the skin, that initially appear small and pink and will eventually harden, leading to a diagnosis of scleroderma. The outlook is excellent for children with morphea, because these patches will eventually soften and fade, leaving no serious, long-term complications.
Linear scleroderma presents as lines of tight, shiny and often darkened skin. These often appear on the child's arm or leg and extend to the hands or feet. It can also affect the underlying muscle, fat and bone, resulting in a scarred appearance. Early diagnosis and treatment for linear scleroderma are very important for growing children, especially if an affected patch of skin crosses over a joint, because normal growth and development can be affected.
Children with linear scleroderma will often have a positive outlook, provided that they receive appropriate and prompt treatment to minimise any longer-term complications.
Linear scleroderma en coup de sabre
Coup de sabre arises when children have linear scleroderma presenting on their face and possibly their scalp. If the affected area is confined to the scalp and forehead then the problem is mainly cosmetic.
Parry Romberg syndrome
This type of scleroderma features similar skin lesions to coup de sabre, but may involve an entire side of the child's face and in some cases the tongue. When one side of the face is affected, there may be additional complications because the facial bones may not grow properly.
The treatment for localised scleroderma usually involves anti-inflammatory medication, physiotherapy and occupational therapy.
Systemic sclerosis in children
A very small number of children are diagnosed with 'systemic sclerosis', the more serious form of scleroderma. In systemic sclerosis, both the skin and internal organs are involved. Of all children diagnosed with scleroderma, fewer than 10% will have this form of the condition.
Systemic sclerosis can appear at any age. Before puberty it occurs equally in boys and girls; however after puberty it arises more frequently in girls.
Children with systemic sclerosis usually have Raynaud's syndrome. Internal organ involvement can lead to symptoms such as difficulty in swallowing and heartburn. Sometimes the heart, lungs or kidneys can be affected, which may require specific tests and treatment.
As with localised scleroderma, treatment usually involves medication and physiotherapy, however children with systemic sclerosis will need expert medical care and long-term support into adulthood.
Coping with scleroderma in children
Scleroderma can have a considerable impact upon children and their families. It may affect a child's ability to do physical activities and there may be numerous hospital appointments to attend, with school hours missed as a result.
An affected child may have to deal with pain, taking medication, exercises and stiffness. They may also face the side effects of their medications, such as mood swings and weight gain from taking steroids.
At school, they may feel uncomfortable about the difference in their appearance and physical abilities. The input of a clinical psychologist is helpful for some affected children and families.
Children should always cover up and wear total sun block to protect their skin from the sun. Because the affected skin in localised scleroderma does not go brown in the sun, it looks more obvious with a tan
Teenagers can become very skilled with camouflage make-up to disguise their affected skin, whether this is on the face or the limbs; and girls may prefer to wear trousers, particularly at school