Factors other than sex, such as race and ethnic background, may influence the risk of getting scleroderma, the age of onset, and the pattern or severity of internal organ involvement. The reasons for this are not clear. Although scleroderma is not directly inherited, some scientists feel there is a slight predisposition to it in families with a history of rheumatic diseases. Doctors say that there are more exceptions than rules to scleroderma - even more so than other diseases. The first step is to get a proper diagnosis, including which form of the disease the child has. The localized form rarely, if ever, turns into the systemic form. While scientists do not yet know what causes scleroderma, it is known that it involves an overproduction of collagen.
Most patients do not have any relatives with scleroderma and their children do not get scleroderma. Research indicates that there is a susceptibility gene which raises the likelihood of getting scleroderma, but by itself does not cause the disease.
There are three types of localized scleroderma: morphea, linear scleroderma, and scleroderma en coup de sabre. Of these, morphea is the most common. It consists of irregular patches of skin that appear on the arms, legs or body. These patches usually start out small and pinkish in color and are not noticeable until they reach the size of a penny. Since many superficial skin infections or irritants can cause pink lesions, a variety of creams can be tried begfore the child is referred to a dermatologist. However, over time, the center of the lesions may become pale, dry and hard. This often prompts a referral to a dermatologist and a diagnosis of scleroderma. In most cases, these lesions are primarily cosmetic. Morphea does not usually appear on the face, and most are easily hidden under clothing. As a result, morphea is more of an emotional issue. The long term prognosis for children with morphea is excellent. Initially the lesions may continue to enlarge and increase in number, however, they will soften and darken over a period of years.
Linear scleroderma is a condition where the areas of skin involvement seem to spread out along lines. Instead of a round area of skin, like with morphea, there may be a streak of involved skin. The involved skin can be on the arm, leg, hand, foot, chest or abdomin. Some children have both linear scleroderma and morphea. Areas of linear scleroderma that cross a joint, such as the elbow, wrist, shoulder, knee, ankle, or finger, can cause permanent damage. As the skin becomes tight, and the lesions harden, they can limit the ability of the joint to move. In a young child, when large areas are involved, the arm or leg may not grow appropriately. Some children with linear scleroderma also have muscle inflammation, called sclerodermatomyositi. The prognosis for children with this type of localized scleroderma is also excellent, provided that they receive the appropriate treatment. There still could be permanent damage if there was either no treatment, or the disease did not respond to the treatment.
Linear scleroderma en coup de sabre is the diagnosis when children have linear scleroderma on the head and/or face. These children sometimes have a deep furrow along the scalp with tight, hard skin that often extended onto the forehead. Sometimes, as in the case of children that have Parry Romberg syndrome, in addition to the lesions seen in linear scleroderma, may have involvement of the whole side of the face, and sometimes the tongue. The prognosis for this type of scleroderma is mixed. If the lesions are confined to the scalp and forehead, they often evolve similarly to linear scleroderma and the effect is mostly cosmetic. The same is true for isolated areas of involvement on the face. However, Parry Romberg syndrome can present mre serious difficulties because the bones may not grow properly. It is very important for parents to remember that involvement of the face is going to be obvious to both the child and to others. These children need extra support in understanding that they did not cause their conditon and in learning how to deal with the disease, and with others.
Appropriate treatments remain controversal, and, because the disease is relatively rare, there are few studies being done. For children with mild morphea, topical treatment with calcipotriene cream or ointment during the active phase is often sufficient. Linear scleroderma usually responds well to methotrexate, and may also be used for children who have widespread morphea. Linear scleroderma that does not cross a joint line usually does not require any treatment. However, when large areas of an extremity are involved, or the disease is crossing a joint line, there is significant risk of permanent damage. In these cases most specialists will recommend methotrexate, which can slowly soften the involved skin. Like all other medications, methotrexate has possible side effects, so careful consideration must be given to the expected risks and benefits. The use of methotrexate for children with linear scleroderma en coup de sabre is still controversial.
For children with the systemic form of scleroderma, which again, is extremely rare, the prognosis is not as good. Juvenile systemic sclerosis (JSS) is a multisystem connective tissue disease characterized by skin fibrosis and internal organ involvement. Skin and vascular manifestations are the most common clinical features, while internal organ involvement is is more rare. Cardiopulmonary disease is the most frequent visceral involvement, leading to significant morbidity. This girl, who is nine years old, has had systemic sclerosis for five years. Tightening of the skin is obvious, and she is unable to close her lips. In addition to cardiovascular involvement, the disease can also involve the respiratory system, the gastrointestinal system, musculoskeletal, and renal function.
For children with diseases, the psychological effects can be more devastating than the physical. Children with chronic illness will very often wonder what they did wrong. Open discussion and reassurance among family members may be sufficient, but sometimes professional help is warranted. No one should be afraid or ashmaed to seek the help tht they need to deal with the stress of chronic illness in themselves or a family member. If allowed to smolder, the stresses of chronic illness can lead to noncompliance by the patient and the can even worsen the disease outcome. Divorce, suicide, and other negative behaviors can all occur with increased frequency, not only in the children themselves, but also in all of their family members.
Donations are needed in order to increase the amount of research for this heartbreaking disease. Please go to http://www.jsdn.org/ to learn more about this disease and to help the cause. This worldwide non-profit children's organization is based all on volunteers. Zero funds go to administrative costs, and this organization does not receive any government funds. They rely on the support of private donations. Please give to end the suffering of children living with juvenile systemic sclerosis.
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