Juvenile Dermatomyositis (JDMS)--What Are teh Symptoms?

There are three main symptoms of JDMS: skin rash, weak muscles, and fever. The skin rash is usually first, and weak muscles follow, usually very slowly and quietly rather than suddenly. Some children may also have other symptoms such as sore or swollen joints and fever. Almost all children with JDMS have less energy and do fewer activities or just refuse to do certain tasks because they know they cannot do them successfully. Many parents of children with JDMS blame themselves for not noticing weakness earlier, they feel guilty about thinking or even worse, saying, that their child is lazy or slow. This is very common. Many physicians fail to recognize this illness during its early stages; it is therefore not surprising that it goes unrecognized, often for weeks or months.

Remember: JDMS can be mild in some children and severe in others. Your child may not have all of these symptoms, and may have only minor problems with some of them. For example, some children with JDMS may only complain of inability to play on the monkey bars while others may become weak so quickly as to be unable to rise from bed or stand up from floor.

MAIN SYMPTOMS

Skin Rash

The skin rash usually occurs on the face, knuckles, elbows, knees, and ankles. In some children, the rash may cover the whole body. The rash usually appears before muscle weakness becomes apparent but it may occur after or at the same time as the muscle weakness. The rash may be made worse by sunlight.

What you may notice: Your child's eyelids and cheeks may appear red or purplish, and the eyelids may become puffy. Red patches that look like dry skin appear over the knuckles, elbows, and knees. The cuticles may appear red and swollen as though the child has been biting them. Because of the puffy eyelids and skin rash, this part of the illness can look very much like an allergy and is often diagnosed as such. The dry rash over knees and elbows may be misdiagnosed as eczema. Since the rash may be made worse by sunlight, children should wear sunscreen when they are in the sun.

Weak Muscles

The body's central muscles, those that are in and near the trunk, such as the neck, shoulders, back, abdomen, and hips will slowly become very weak. The muscles used for swallowing and breathing may also become weak. This happens after the central muscles are already affected and could be dangerous and be the reason the child with JDMS is admitted to the hospital.

What you may notice: Your child is likely to have trouble with common movements such as climbing stairs, climbing into a car or a bus, riding a bicycle, or getting out of a bathtub. It may also be difficult to get up from the floor or from a low chair. Your child will probably have a hard time keeping up physically with children the same age, may fall more often than usual, and may often feel tired. Children with JDMS may also complain that their muscles are sore to touch.

In very severe cases of JDMS, even the muscles used for swallowing can be affected. Your child may have trouble swallowing chunks of food such as meat and may choke on some foods. These same muscles are used for speaking, so your child's voice may become weak, and she may sound as if she is talking through her nose or simply run out of voice. If you or your child notice any of these symptoms, contact the doctor immediately.

Fever

At the start of JDMS, some children have a fever along with the other symptoms described above.

What you may notice: Your child's temperature will rise one or two degrees.

OTHER POSSIBLE SYMPTOMS

The following symptoms may also occur in JDMS. Some are less common, but since they can occur you should know about them.

Stiff and Swollen Joints

In JDMS, one or several joints may become stiff or inflamed (swollen). The joints feel stiff or sore because the muscles around them are inflamed, or because the joint itself is inflamed. Unlike other forms of childhood arthritis, the inflammation usually doesn't last long or cause joint damage.

What you may notice: Your child may complain of stiff and sore joints. The joint may also feel a bit warm when you touch it, it may be swollen and even red.

Vasculitic Ulcers

A vasculitic ulcer is a hole in the tissue that surrounds an inflamed blood vessel. In JDMS, vasculitic ulcers can occur in the skin and in the bowels (the gastrointestinal tract through which food travels from the esophagus to the rectum).

In the skin: These ulcers happen when the blood vessels under the skin are severely inflamed. They look like sores in the skin rash, and can be as small as a pencil point or larger than a silver dollar. Some ulcers are very deep, while others occur only on the surface. They can be very painful, and can last anywhere from a few days to several months. The ulcers usually go away with treatment.

In the bowels: These ulcers happen when the blood vessels in the protective lining around the bowels are inflamed. This causes a break, or hole, in the lining. Strong digestive juices can then leak through that hole and damage digestive organs. If this happens to your child, you may notice these symptoms:

severe pain in the abdomen
coal-black stools
change in pattern of bowel movement
passing blood in stools.

This happens only rarely, but if you or your child notice any of these symptoms contact the doctor immediately.

Calcinosis

Calcinosis (kal-sih-NO-sis) is a process that causes small lumps of calcium to form under the skin or in the muscle. The lumps may feel like little rocks under the skin and can range from the size of a period on this page to the size of a flat pebble. Sometimes, the smaller lumps join together to form larger calcium lumps. When this happens, it can impair muscle movement. The lumps are not caused by too much calcium in your child's diet - they are a late result of the illness and occur in more than half of all children with JDMS. Calcium lumps are usually not present at the beginning of the illness. It is thought that calcium is more likely in children who have untreated muscle inflammation either because they were not diagnosed in time or were not treated aggressively enough to control inflammation promptly. It is possible however that no matter what is done or not done, some children with JDMS are at risk for calcinosis as part of their particular illness.

If the calcium lumps are bumped a lot, they can become sore. This is most likely to happen if they are on your child's knee, elbow, buttocks, or other pressure joint. Small pieces of creamy white calcium may break through the skin and drain from the hole. These draining areas will usually heal, but they can become infected. Contact your doctor if this happens.
In most children, after a while, the calcium lumps are usually absorbed back into the body. Rarely, the calcinosis becomes extensive and significantly affects movement.

Contractures

A contracture is a shortened muscle that causes a joint to remain in a bent position. In JDMS, there are two ways a contracture may occur:

Through the healing process: As the muscle heals, it may scar. These scars can shorten the muscle, and pull the joint into a bent position.

Through a lack of exercise: At times, your child's muscles might feel too sore to exercise. Without exercise, the muscles become weak. Because a weak muscle cannot hold the joint in its correct, extended position, a contracture will occur.

You and your child can help prevent serious joint contractures with daily exercises and proper physical therapy.

Slowed Growth

When the body is fighting a disease, it uses a lot of the energy which it would normally use for growth. Because your child may not feel well, she may not feel like eating. These factors, along with some of the medicines used in treatment, may cause your child to grow and mature more slowly than normally. Usually, your child will begin to grow again once the disease is under control.

NOTE: Juvenile dermatomyositis (JDMS) affects 3,000 to 5,000 children in the United States. While certain signs and symptoms are common in most children with JDMS, the disease affects different children in different ways. The following information describes the general symptoms of and treatment for JDMS, but it is important for you to discuss your child's particular case with your doctor.