Childhood Scleroderma

Scleroderma in childhood is rare affecting less than 3% of all cases referred to specialist paediatric rheumatology services but those affected have particular problems, especially related to growth. Skin lesions called localised scleroderma are the most common in children. There are two types of localised scleroderma, morphea and linear scleroderma. However, both can be found in the same child, and may change from one type to the other over time. Localised scleroderma lesions do not suddenly appear, but develop gradually over many years. A small number of children have their disease complicated by active arthritis on the affected side, including tenosynovitis. It would appear that occasionally linear scleroderma seems to start after a minor injury, while both the linear and morphoea have been described as developing after viral infections such as chicken pox. Otherwise the cause is unknown and efforts to incriminate a single organism, such as that for Lyme disease, have proved negative.

Morphoea

Morphoea affects a local area of skin, usually the trunk or limbs. It often starts as a purplish area, which gradually becomes pale and thickened, sometimes keeping a purple coloured edge. This skin is tight and waxy to touch. Morphea is usually a single lesion which may gradually increase in size over a number of years. Other lesions may appear, often in the same area, and rarely morphea can cause widespread skin changes. Eventually most morphoea lesions become inactive and scarred in appearance.

Linear Scleroderma

As the name suggests the skin affected is in a line, usually along an arm or leg. The skin may feel tight, with a loss of the normal plumpness of the skin, which becomes shiny, often ivory coloured, or sometimes darkened.

Linear scleroderma can also affect the underlying muscle, fat and bone leaving a scarred appearance. This is important in growing children, as normal growth may be reduced in the affected area. This can eventually lead to the affected arm or leg being shorter than the unaffected side. It is also important if the lesion crosses over a joint such as an elbow or knee. The tightening of the skin may prevent the joint from straightening or bending fully and the joint can become fixed in one position. This is called a contracture. Sometimes linear scleroderma can occur on the face, and may result in a scar just like that from a sword cut, and so is called ‘en coup de sabre’. Sometimes the rest of the fat, muscle and bone on the same side of the face can waste away (atrophy) leaving a pinched appearance to one side of the face (hemifacial) called ‘hemifacial atrophy’. This is also sometimes called the ‘Parry Romberg Syndrome’.

Other forms of childhood scleroderma

A very small group of adolescents, usually girls aged thirteen to fourteen, develop Raynaud's and rapid tightening of the skin, particularly over the hands and feet with creaking tendons. Some may have difficulty in swallowing or shortness of breath. The majority seem to improve over a period of time, particularly with the use of physiotherapy, non-steroidal anti-inflammatory drugs and penicillamine.

A few children develop nodules along the tendons before the skin change is obvious, presenting with difficulty in using wrists or fingers. This may be mistaken for juvenile arthritis, unless patches of local skin involvement, either morphea or linear, are noted. Physical and occupational therapy to maintain muscle strength and range of movement is important for both these groups.

HOW IS THIS MANAGED?

In the management of children, it is important to document the lesions carefully. This will usually be recorded by measuring the size of the lesion, the length of the affected limb and the length of the other limb.

To download our leaflet on Childhood Scleroderma click here
To download our leaflet on Raynaud's in Teenagers and Youngsters click here
To visit the British Paediatric Surveillance Unit (BPSU) website click here